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British Library Cataloguing in Publication Data: Fletcher, Christopher D.M. An atlas of gross pathology. 1. Pathology I. Title II. McKee, Phillip H. 616.07 RBll1 Project Editor: Michele Campbell Design: Nigel Duffield ISBN: 0-906923-47-6 (Gower) 0-7131-4557-9 (Arnold)
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Preface
•
•
The aim of this atlas is to provide an introduction to the macroscopical appearances of the most common pathological conditions for undergraduate medical students and nurses in training. It will also, hopefully, be of value to postgraduates undertaking the FRCS examinations, for whom a working knowledge of gross pathology is vital. Only important or frequently encountered disease processes are covered. Each illustration is accompanied by a concise legend outlining basic, relevant clinicopathological and pathogenetic details. In collecting material for this atlas, we are deeply indebted to Professor J.R. Tighe of the Histopathology Department at St. Thomas's Hospital for allowing us access to the departmental collection of colour transparencies. Weare also particularly grateful to Dr H. Pambakian, Museum Curator at St. Thomas's Hospital Medical School and Professors H. Spencer and M.S.R. Hutt. Most of all, this book would not have been possible without the consistent generosity and thoughtfulness of all the pathologists in our department, who unselfishly offered us many of their specimens, obtained either surgically or at post mortem, for photography. . COM Fletcher & PH McKee London
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111 .
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Acknowledgements ,
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•
IV
The authors would like to thank the following colleagues for providing illustrative material: Professor P.G. Bullough, Cornell University Medical College, New York (Figs.7.16 , 12.1 bottom, 12.4, 12.7-12.10,12.13-12.15,12.17,12.18, 12.20-22, 12.24, 12.25 & 12.29 top); Dr D.W. Day, Dept. of Pathology, University of Liverpool Medical School, Liverpool (Fig.3.23 ); DrC.W. Elston, Dept. of Pathology, City Hospital, Nottingham (Fig.9.35); Profossor P.L. lantos, Dept. of Neuropathology, Institute of Psychiatry, London (Figs. 11. 10, 11.20 & 11.24); Dr].C. Macartney, Dept. of Histopathology, St Thomas's Hospital Medical School, London (Figs.4.26, 4.29, 7.8 & 12.26); Professor F.V. O'Brien, School of Dentistry, Queen's University, Belfast (Figs.3.1 & 3.2); Dr C. Parkinson,"Institute of Urology, London (Figs. 10.7 & 10.13); DrD.E. Sharvill, William Harvey Hospital, Ashford, Kent (Fig. 5.13 ); Dr ].M. Sloan, Senior Lecturer/Consultant Pathologist, Royal Victoria Hospital, Belfast (Figs.2.3, 2.4, 2.14,8.24 & 8.30); The Wellcome Museum, Royal College of Surgeons of England, London (Figs.8.20 & 9.28).
Contents III
Ii 1IIIIwing
Preface
iii
Acknowledgements
iv
1 Cardiovascular System 2 Respiratory System
1
3 Alimentary System 4 Hepatobiliary System 5 Breast 6 Lymphoreticular System 7 Endocrine System 8 Urinary System 9 Female Reproductive System 10 Male Reproductive System 11 Nervous System 12 Osteoarticular System Index
13
23
36
45
50
55
62
72
83
87
94
103
.... I
I
v
1 Cardiovascular System
Fig.1.1 Recent myocardial infarct. A transverse section through both right and left ventricles, viewed from below. The anterior wall of the left ventricle shows an extensive area of recent infarction, charac terised by an almost full-thickness zone of yellow necrotic myo cardium, surrounded by a hyperaemic rim . The latter consists of granulation tissue (capillaries and fibroblasts) and represents the early phase of healing. This infarct is of approximately one week's duration. In nearly all cases, myocardial infarction is caused by occlusive thrombosis in an atheromatous coronary artery. Rare causes include syphilitic aortitis, polyarteritis nodosa and coronary artery embolism from a variety of cardiac lesions .
Fig.1.2 Healed myocardial infarct. The heart has been opened to display the inner aspect of the left ventricle . Marked pale fibrous scarring is seen in the posterior wall and in the papillary muscles. Mural thrombus overlying the scar is also present. Healing, by fibrosis, commences about 3 weeks after acute infarction and is usually complete after 2 months . Fibrous replacement of the myo cardium predisposes to aneurysm formation (see Fig .1.6) within 'vhich thrombus may form.
1
Fig.1.3 Coronary artery thrombosis. The left main stem coronary artery has been opened longitudinally to reveal occlusion of its lumen by thrombus (arrowed). Note the presence of atheroma in the ascending aorta and a fibrinous pericarditis. Occlusive coronary thrombosis almost always occurs at the site of an atheromatous stenosis (see Figs.1.36-1 .38) and is thought to be initiated either by ulceration or haemorrhage into a plaque. Fig.l.4 Coronary artery thrombosis. The left anterior descending coron ary artery is shown in transverse section . The lumen is mark edly diminished by atheroma, and over lying thrombus has resulted in total occ lusion . In the vast majority of cases of myocardial infarc tion, such occlusive thrombosis will be detected if the coronary arteries are examined with suffi cient care.
Fig.1.5 Myocardial infarct with mural thrombulI '''''' ~ rupture. The heart has been opened to expo:'!!) III" 'jill ,I I left ventricle. A large mural thrombus is adhOlull1 Il ' lLI l !Il ' myocardial infarction, complicated by rupture (111I1I ! illli ll septum. The probe has been passed through II" , It 11110 II ' extreme left of the pictu re its tip can be seen IIV' !lIYII II I II " ventricular flap. Myocardial rupture, which is not III II "II it ' occurs within a week of acute infarction,
Flg.l.S Left ventricular aneurysm. The d(~ vO IIlI'"1I 11 11 , aneurysm of the left ventricle is a not uncomllliJlt 111 111 1 III ' myocardial infarction. It is due to replacemellt (11111 ) I"Y" collagenous scar tissue with resultant loss of 0111;, 111 il y 'II aneurysms often contain mural thrombus whic:h " Illy I II systemic emboli. The laceration of the anterior' Ili lllllllllY " right of the aneurysm occurred during the post 1111111,,,,,
1 Cardiovascular System
left main stem coronary occlusion of its lumen of atheroma in the . Occlusive coronary of an atheromatous to be initiated either by
Fig.1.4 Coronary artery thrombosis. The left anterior descending coron ary artery is shown in transverse section. The lumen is mark edly diminished by atheroma, and over lying thrombus has resulted in total occ lusion . In the vast majority of cases of myocardial infarc tion, such occlusive thrombosis will be detected if the coronary arteries are examined with suffi cient care .
Fig.1.5 Myocardial infarct with mural thrombus and ventricular rupture. The heart has been opened to expose the septal wall of the left ventricle. A large mural thrombus is adherent to an area of recent myocardial infarction, complicated by rupture of the interventricular septum. The probe has been passed through the rupture and at the extreme left of Ihe picture its tip can be seen overlying the righl vent ricular flap. Myocardial rupture, which is not uncommon, usually occurs within a week of acute infarction .
Fig.1.7 Haemopericardium. The pericardial sac has been opened (left) to show an extensive haematoma overlying the epicardium. On the right, the haematoma has been removed to reveal the cause as being a slil -like ventricular perforation (arrowed) at the site of a recent myocardial infarct. Haemopericardium may more rarely occur as a complication of dissecting aortic aneurysm or trauma.
Fig.1.6 Left ventricular aneurysm. The development of an aneurysm of the left ventricle is a not uncommon late complication of myocardial infarction. It is due to replacement of the myocardium by collagenous scar tissue with resultant loss of elasticity. Such aneurysms often contain mural thrombus which may be a source of systemic emboli . The laceration of the anterior papillary muscle to the right of the aneurysm occurred during the post mortem.
Fig.1.8 Ruptured papillary muscle. The heart has been opened to display the posterior aspect of the left ventricle . In the centre of the picture is a portion of the anterior papillary muscle which has been , torn and shows obvious necrosis. Rupture of a papillary muscle is a rare complication of myocardial infarction, which usually occurs within 2 weeks of the primary event: it results in the acute onset of mitral incompetence and left ventricular failure.
2
1 Cardiovascular System
Fig.1.9 Left ventricular hypertrophy. Left ventricutar hypertrophy is a not uncommon finding at post mortem owing to the frequency of essential hypertenSion in the population. A list of causes is given in Fig:1.10 In this instanc e the increased thickness of the left ventricular wall is obvIous (in excess of 20mm) However, a much more accurate method of assessing venlricular hypertrophy involves weighing the chambers separatel y after careful dissection, thereby taking into account any degree of associated ventricular dilatation .
Fig.1.11 Acute rheumatic endocarditis. Characteristic small pink vegetations (arrowed) are present along the line of closure of this mitral valve cusp. Rheumatic fever, a multisystem autoimmune process, is a rare complication of (3-haemolytic (Group A) strepto coccal infections . It results from the development of heterophilic cross·reacting antibodies to the streptococcal M protein and an, as yet unidentified, connective tissue antigen . Manifestations include a pancarditis, joint involvement , skin rashes, subcutaneous nodules and , rarely, Sydenham's chorea .
Fig.1.13 Mixed mitral valve disease. Th erl; 1: , II,.lfl" , II , the chordae tend inae wilh fusion and short e"" ,i I 11" , III' process has produced a rigid 'buttonhole' va lv,' II" ,jO . j 'I stenotic and incompetent - the latter has resulu )d '" II I'" II of left ventricular hypertrophy. as seen in th e Ix ,II", II ' If I1 I1 corner.
CAUSES OF MITRAL INCOMPETENCr
CAUSES OF LEFT VENTRICULAR HYPERTROPHY Systemic hypertension
Rheumatic heart disease
Aortic stenosis Aortic incompetence
Papillary muscle rupture or fibrosis
Mitral incompetence coarctation of aorta
Congenital heart disease
Congenital
reversed VSD
Amyloid
Mitral valve prolapse (floppy valvo !,Y'ldJl '" I,
Cardiomyopathy anaemia
Functional dilatation of valve ring
thyrotoxicosis
High output failure
Paget's disease A-V malformation
-
-
-
-
-
-
Fig.1.10 Causes of left ventricular hypertrophy.
3
-
-
Fig.1.12 Mitral stenosis with atrial thrombus. The commonest complication of rheumatic endocarditis is mitral stenosis and , indeed , almost all stenotic mitral valves are of rheumatic origin . Fusion of the valve cusps and fibrosis results in narrowing of the valve orifice. The stenosis causes leli atrial dilatation and may be complicated by atrial fibrillation with consequent thrombus formation , as seen in this case.
Marfan's syndrome I
.Fig.1.14 Causes of mitral incompetence.
1 Cardiovascular System
Fig.1.13 Mixed mitral valve disease. There IS marked fibrosIS 01 the chordae tendinae with fusion and shortening. The rheumatic process has produced a rigid 'buttonhole' valve, thereby being both stenotic and incompetent· the latter has resulted in the deve lopment of lelt ventricular hypertrophy, as seen in the bottom right hand corner.
I
CAUSES OF MITRAL INCOMPETENCE
Fig .1.15 Aortic stenosis. Isolated aortic stenosis may comp licate rheumatic heart disease but more often is associated with mltrat involvement also. The proximal portion of the ascending aorta has been opened to view thi s stenotic valve from above. Aortic stenosis usually gives rise to left ventric ular hype rtrophy and may compromise the coronary blood supply.
I
Rheumatic heart disease Papillary muscle rupture or fibrosis Congenital Mitral valve prolapse (floppy valve syndrome)
Functional dilatation of valve ring
Marfan's syndrome Fig.1.14 Causes of mitral incompetence.
Fig.1.16 Calcific aortic stenosis. Calcification of the aortic val ve most commonly occurs in a congenital bicuspid valve, but may also arise as a consequence of rheumatic disease and is sometimes a feature of the ageing process. Note the coa rse ca lcifi c nodules in the va lve cusps
4
1 Cardiovascular System
r-
CAUSES OF AORTIC STENOSIS
I
TYPES OF ENDOCARDITIS bacterial
Rheumatic heart disease
viral of congenital bicuspid valve Infective
Calcification
rickettsial chlamydial
senile
fungal dome-shaped valve
Rheumatic Congenital
supravalvar stenosis
Non-infective thrombotic (agonal) Libman-Sacks (S.L.E.)
subvalvar stenosis
Fig.1 .19 Types of endocarditis.
Hypertrophic cardiomyopathy
I
Fig.1.21 Infective endocarditis (normal valve). VIII 11.1 I present on all three cusps of th is otherwise nOIIlliII.II 1111< ~ Endocarditis affe cting normal valves is usually <111 11 11 < I disease aHecting immunocompromised patient:; i ll" 1111 111 the latter group the right side of the heart may lJOIIIVI .lvl" I
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- - - -
Fig.1.17 Causes of aortic stenosis.
CAUSES OF AORTIC
~NCOMPETENCE
J
Rheumatic heart disease Syphilitic aortitis
Congenital bicuspid valve
Marfan's syndrome
Ankylosing spondylitis
I Aortic sinus aneurysm Fig.1.18 Causes of aortic incompetence.
5
Fig.1.20 Infective endocarditis (damaged valve). Infection of the endocardium and valves may be due to a diverse variety of micro organisms (Fig .1. 19) and, while it may affect previously normal tissue, it develops more often in the presence of pre-existing (parti cularly rheumatic) valvular disease. Streptococcus viridans is the commonest cause of infective endocarditis in previously damaged valves but Staphylococcus aureus, I)-haemolytic Streptococci and Streptococcus pneumoniae are the most usual aetiological agents in cases with no evidence of prior disease. Large friable vegetations obscure the underlying fibrosed mitral valve in this case.
Flg.1.22 Non-infective thrombotic endocarditis. I I il l . I which may aHect the aortic and mitral valves, is 0110[1 1" ,1111 patients dying with disseminated malignant tUIIIOIII" ! I" vegetations seen on this aortic valve are similar tn. 01111 I III" confused with, those of rheumatic endocarditis .
1 Cardiovascular System
OF
E~DOCARDITIS
[
TYPES OF CARDIOMYOPATHYHypertrophic (± obstruction)
bacterial viral
alcoholism
rickettsial
!IVII
parturition
chlamydial fungal I
Congestive , may be associated with
beri-beri Friedreich's ataxia
I If I idiv€) thrombotic (agonal)
muscular dystrophies II
Sacks (S.L.E.)
n'"ndocarditis.
Fig.1.21 Infective endocarditis (normal valve). Vegetations are present on all three cusps 01 this otherwise normal aortic valve. Endocarditis affecting normal valves is usually a more fulminant disease affecting immunocompromised patients and drug addicts: in the latter group the right side of the heart may be involved .
Restrictive (endomyocardial fibroelastosis) Obliterative (endomyocardial fibrosis) Loftier's endocarditis -
Fig.1 .23 Types of cardiomyopathy.
ndocardltis (damaged valve). Infection of the ! vlllv'i:. Inny be due to a diverse variety of micro 1' 1).11 III. while it may affect previously normal • " 11 '11' , Iitun
in the presence of pre-existing (parti
I VIl lvrrloi l tii :.;ease. Streptococcus viridans is the " , " ir IIr ,Ltive endocarditis in previously damaged
I",reus. j3-haemolytic Streptococci and IIIPI"I II.'" i1' O the most usual aetiological agents in h'"' 'I ' ,t I ,rr(,l r disease. Large friable vegetations Iy" 'I I I" " 0110(1 mi tral valve in this case. 'I ' " ,
/I ' .
Fig.1 .22 Non-infective thrombotic endocarditis. This condition, which may affect the aortic and mitral valves, is often found in patients dying with disseminated malignant tumours. The pink vegetations seen on this aortic valve are similar to, and may be confused with, those of rheumatic endocarditis.
Fig.1.24 Hypertrophic obstructive cardiomyopathy. A true cardiomyopathy is, by definition, any myocardial disease without an identifiable cause which appears non inflammatory . Hypertrophic cardiomyopathy is typified by asymmetrical left ventricular hyp ertrophy. especially of the septal wall. Most commonly it is familial, the mode of inheritance being autosomal dominant. In this case the septal hypertrophy has led to obstruction of the out flow tract. Hypertrophic obstruc tive cardiomyopathy is a rare but important cause of unexpected sudden death .
6
I Cardiovascu lar Syste m
Fig.1.27 Fibrinous pericarditis. Pericarditis may occasiona lly be due to primary pyogenic infection , but more commonly a fibrinous exudate occurs as a consequence of a variety of disorders including myocardiat infarction (Fig .13), rheumatic le ver , uraemia, connective tissue diseases and adjacent infecti ve conditions. for example bacterial pneumonia (in which instance the exudate may be fibrinopurulent) . In this case a generalised septicaemia has resu lted in a typical 'bread and butter' appearance .
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Fig .1.25 Congestive cardiomyopathy. Congestive cardio· myopathy is defined as congestive cardiac failure with no apparent cause. and re sults in a dilated , flabb y heart. as in this case. This appear ance may be seen in association with alcohol abuse and pregnancy . Fig.1.26 Endo myocardial fibrosis. Endomyo· cardial fibrosis occurs most commonly in tropical Africans and is mani· fest as dense fibrosis of the ve ntri· cular endocardium . A popular hypo· thesis is that the lesion has a viral aetiology . Involve· ment of the papillary muscles may induce va lve dysfunction. Fig.1 .28 Tuberculous pericarditis. The adherent parietal and visceral pericardial membranes have been separated to disp lay numerous small white miliary tubercles on the surface of the latter . Note also the pale fibrous thi cke ning of the parietal layer. Tuber· culous pericarditis is said to result from lymphatic spread of org anisms from adjacent pulmonary or mediastinal foci of infecti on.
7
Fig.1 .30 Left atrial myxoma . The heart 11;1:: 11"",,1 '1",,1 the left atrium and mitral valve. AttacherJ to 11,1 ' III ",1. " 11 11 left atrium is a large, pedunculated multiloiJld:! 11111 111111' myxomas are rare and clinically may pre sen t w,II" JlIlIi ., II emboli, or occaSionally, with acute pulmon;IIY 1)0" I. ""., ,I truction of the mitral valve orifice . Their prc!:"'" " "",, . " uncertain since there is argument as to wlloll" 'I II" 'Y 01 '1' 'neoplasms or are simply organised thron,1 JI
1 Cardiovascular System
Fig.1.29 Cons trictive pericarditis. This is a very rare disease in which the heart becomes encased by a densely adherent, thickened and fibro· tic pericardium . The aetiology is often uncertain. Whi le some cases may be due to tuberculous infection. occasion· ally a collagen disease is implicated . Fig.1.31 Lambl's excrescence. The occurrence of small fibrin deposits on the heart valves is not an uncommon finding at post mortem Occasionally they may acquire tumour·like appearances, as seen in this case. The aetiology is uncertain.
Fig.1.30 Left atrial myxoma. The heart has been opened to display the left atrium and mitral valve. Attached to the posterior wall of the left atrium is a large , pedunculated multilobular tumour. Atrial myxomas are rare and clinically may present with multiple systemic emboli, or occasionally , with acute pulmonary oedema due to obs· truction of the mitral va lve orifice . Their precise nature is , as ye t, uncertain since there is argument as to whether they represent true neoplasms or are simply organised thromb i.
Fig.1.32 Epi cardial secondary deposits. This heart was taken from an elderly male dying from squamous ce ll carcinoma of the bronchus . The epi· cardium is covered by small pale umbilicated metastases. In addition there is a large tumour mass situated between the superior vena cava and the pulmonary trunk, seen at the top of the picture (arrowed). This resulled in vena caval obstruction. Epicardial or peri· cardial tumour metastases often induce a fibrinous or fibrinopurulent pericarditis .
8
1 Cardiovascular System
Fig.1.33 Brown atrophy. This heart, which was removed from an 86·year-old woman with senile dementia, is very small and weighed only 180g (normal adult female 250-300g) . Note the brownish dis colouration. This change is an ageing phenomenon, in which lipo fuscin pigment, representing the lipid remnants of effete organelles, is deposited in many organs in association with atrophic changes, probably due to a combination of relative ischaemia and disuse . Flg.1.34 Myo cardial fatty degeneration. Acc· umulation of lipid within the myo cardium may occur in a variety of con ditions including severe anaemia, alcohol abuse and poisoning. As seen in this papillary muscle, it produces a characteristic 'thrush breast' appearance.
9
Fig.1.35 Fatty streaks (aorta). This aorta has been removed from a small child and stained by the Oil Red 0 technique to demonstrate lipid . Small intimal depo· sits are seen . The orifices of the inter costal arteries are on the left. Such juvenile fatty streaks are found in the large arteries of children and adolescents of all races and socio· economic groups. It is unlikely that these lesions bear any pathogenetic rela tionship to the future development of atheromatous plaques . Fig.1.36 Uncomplicated atheroma. This section of aorta, from a middle-aged male, shows numerous raised, irregular intimal deposits. Atheroma is the commonest cause of death in the Western World , largely by giving rise to myocardial infarc tion and cerebrovascular acci· dents. Known risk factors for its development include increasing age, cigarette smoking, hyper· tension, a diet high in saturated fats, hyperlipidaemia and diabetes mellitus. Its exact pathogenesis is unclear, but intra-intimal lipid deposition and incorporation of mural thrombi are popular hypotheses. Myo fibroblasts within plaques have been shown to be monoclonal in origin, the significance of which is uncertain.
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1 Cardiovascular System Fig.1.35 Fatty streaks (aorta). This aorta has been removed from a small child and stained by the Oil Red 0 technique to demonstrate lipid . Small intimal depo sits are seen. The orifices of the inter costal arteries are on the left. Such juvenile fatty streaks are found in the large arteries of children and adolescents of all races and socio economic groups. It is unlikely that these lesions bear any pathogenetic rela tionship to the future development of atheromatous plaques. Fig.1.36 Uncomplicated atheroma. This section of aorta, from a middle-aged male, shows numerous raised, irregular intimal deposits . Atheroma is the commonest cause of death in the Western World , largely by giving rise to myocard ial infarc tion and cerebrovascular acci dents. Known risk fac tors for its development include increasing age, Cigarette smoking , hyper tension, a diet high in saturated fats, hyperlipidaemia and diabetes mellitus . Its exact pathogenesis is unclear, but intra-intimal lipid deposition and incorporation of mural thrombi are popular hypotheses Myo- . fibrob lasts within plaques have been shown to be monoclonal in origin , the significance of which is uncertain .
Fig.1.37 Ulcerated atheroma. Necrosis within an atheroma tous plaque, in combination with constant haemo dynamic stress may lead to ulceration, as seen in this picture . As a consequence, exposure of sub endothelial tissues may result in thrombus formation . This in turn may cause vascular occ lusion of medium and small arteries , as seen in the coro nary vessels (Figs.1.3 and 1.4).
Fig.1.38 Compli cated atheroma. This segment of aorta has been opened to show all the major complica tions that may occur in atheromatous plaques. There is extensive ulceration, haemorrhage and dystrophic calcifica tion; scattered small thrombi are present, overlying some of the lesions. It is not uncommon for frag ments of such comp licated plaques to break off and pro duce systemic emboli, the conse quences of which will clearly depend on the site of the affected artery
Fig.1.39 Syphilitic aortitis. The left ventricle and ascending aorta have been opened to show the characteristic irregular 'wood bark' appearance of the aortic intima. The aortic valve cusps are rolled and thickened and there is separation of the commi ssures. Syphilitic aortitis is the commonest manifestation of ter tiary infection and usually affects only the intrathoracic portion of the aorta. It results from a florid arteritis of the vasa vasorum with consequent intimal fibrosis . Common complications of th is condition include aortic incomp etence, aneurysm formation and stenosis of the ostia of the coro nary arteries.
Fig.1,40 Polyarteritis nodosa. The epicardium has been stripped from this heart to demonstrate branches of one of the coronary arteries, which show fibrosis of the vessel walls and formation of several small saccular aneurysms. Polyarteritis nodosa is an uncommon disease. thought to be due to immune complex deposi tion and sometimes associated with hepatitis B or systemic lupus erythematosus. It affects small arteries and arterioles in any part of the bod y and results in inflammation and necrosis of the vessel walls , commonly with overlying thrombosis. The cl inical effects are largely due to ischaemia of the affected organs. Small aneurysms develop quite often and may be compl icated by rupture .
10
1 Cardiovascular System Fig.1.43 Abdominal aortic aneurysm. The lower portion of the abdominal aorla has been opened to show a saccular dila tion of the distal end, the lumen of which contains a large lamina ted thrombus . Proximally, the aorta shows extensive involve· ment by complicated atheroma . This is the commonest variet y of true aortic aneurysm in the Western World and is almost always a consequence of exten sive atheroma, leading to thinn-· ing or disruption of the aortic media. Older adults, particularly males, are often affected. Such aneurysms may rupture with extensive retroperitoneal haem orrhage and this may be fatal. The formation of large intra luminal thrombi sometimes gi ves rise to aortic occlusion with distal ischaemia or to embolic phenomena .
Fig.1.41 Deep venous thrombosis. The femoral v ein has been exposed in a patient dying of pulmonary embolism (see Fig.2.16). The lumen of the vein is vi rtually occluded by thrombus which is adherent to the endothelial surface. In general terms the pre· disposing faCtors to thrombosis are encompassed in Virchow's triad (1) alteration in the vessel wall , (2) alteration in the blood flow and (3) alteration in the blood constituents . Deep ve nous thrombosis is commonest in the calf veins and , as in th is case, may be complicated by pulmonary embolism, although the frequency with whic h this occurs is diffic ult to determine since many venous Ihromboses go undetecte d. The co mmonest causes include immobility, myocard ial infarction, pregnancy or childbirth, varicose veins or phlebitis and any severely debilitating disease such as cancer.
CLASSIFICATION OF TRUE ANEURYSMS
--I
- --
-
Atheromatous Syphilitic I
Infective Cirsoid A-V fistula
I
Berry (cerebral) Charcot-Bouchard (cerebral-hypertensive) I
Erdheim's medial degeneration (dissecting) Marian's disease (dissecting)
J
Fig.1.42 Types of true aneurysm , to be distinguished from a 'false' aneurysm, which represents the site of a walled -off arterial rupture.
11
Fig.1.44 Dissec ting aortic aneurysm. The term dissecting aneurys m is a misnomer in that true dilatation of the vessel wall does not occur. Rather. the apparent increase in size is due to the presence of thrombus wilhin the media of the artery, as seen here in the arch of the aorta . Dissecting aneurysm is most commonly due to mucoid degenera tion (Erdheim) of the media but may also be seen in Marfan's syndrome and in association with systemic hype rtension.
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1 Cardiovascular System
inal aortic vver portion of 'ra has been saccular dila d. th e lumen a large lamina ximally. the sive involve led atheroma. nest va ri ety of minthe 'd is almost en ce 01 exten ding to thinn- f the aortic IS. particularly :f1ected Such Ipture with i tonea l haem ay be latai. rge intra metimes gives sion with distal boli c
44 Dissec o rtic sm. Th e term ling aneurysm i s nomer in that i latation of the I wall does not . Rather. the ent increase in due to the
nceof
bus with 'ln the
of the artery ,
I3n here in th e
) f the aorta
1ing sm is most o nly due to d degenera rdh eim) of the b ut may also ",n in Marfan 's p meand in iationwilh nic hypertension.
Fig .1.45 Dissecting aortic aneurysm. The process of dissec tion is initiated by a transverse intimal tear (ar rowed) . usually In the proximal pari of the ascending aorta (top) . Extens ion of the process c auses formation of a fa lse lumen with in the media which . on transverse section . produces a typical doub le-barrelled appearance (bollom) . Dissecting aneurysm is usuall y fatal either by rupt ure or by retro grade Invotvement of the co ronary arteries. Ve ry occasionally the dissec ting process re-enters the true lumen of the aorta .
Fig.1.46 Dissect ing aortic aneurysm . tn thi s instance th e dissec tion has resulted in rupture of the aortic root and the development of haemopericardium External rupt ure may atso occur into the pfeu ral cavity (leading to a haemothora x) or mediastinum .
Fig.1.47 Splenic artery aneurysm . The large sac cular aneu rysm above the bod y of the pancreas was found in a young woman with no evidence of atheroma or syphilis. The red probe demonstrates luminal continuit y between the aneurysm and the distal portion o f the artery. The lesion probably re presents a rare example of a congen ital aneurysm due to fibromuscular displasia of th e arterial wall .
Fig.1.48 Monckeberg's sclerosis. Mbnckeberg' s sclerosis is c haracterised by calci fi catio n of the media 01 the large arteries. particularly in the limbs and pelvis It appears to be part of the normal ageing process and is commone r in diabetics. The diagnosis is usually made radiologically . In th is plain X-ray of a post -mortem specime n of the femoral arteries. the typ ical fine tram-line appearance of the c alcific ation is clearly se en .
12
2 Respiratory System Fig.2.1 Laryngeal squamous carcinoma. The trachea and larynx have been opened poster iorly to reveal a small fungating supraglottic tumour arising in the right aryepiglottic fold . Squamous carcinoma is the commonest malignant tumour of the larynx and arises most often in the 6th and 7th decades, affecting males more frequently than females. Known risk factors include chropic inflammation and cigarette smoking. Spread is largely local or lymphatic and overall 5-year survival is about 65%.
Fig.2.3 Lobar pneumonia. Uniform red , firm consolidation of this left upper lobe, with comp lete sparing of the lower lobe, is typical of 'red hepatisation' - the second stage of lobar pneumonia. This occurs at about the 2nd to 4th days in an untreated patient, being preceded by engorgement and succeeded by 'grey hepati sation' and resolution at about the 8th to 10th day . Lobar pneumonia, particularly in its cfassical form, is rarely seen nowadays with the advent of modern antibiotic therapy . How ever, young adults are most often affected. over 90% of cases being due to
Streptococcus pneumoniae.
Fig.2.4 Lobar pneumonia. There
Fig.2.2 Bronchopneumonia. This left lung shows congestion and diffuse multifocal consolidation (left) . A close-up view from a different case (right) shows small areas of consolidation and suppuration. largely centrilobular in distribution. Bronchopneumonia is principally a disease of the ve ry young and old , but also occurs in immuno suppressed patients. Chronic obstructive airways disease and viral respiratory infections are frequent predisposing factors. A very wide variety of causative organisms may be isolated, of which . Streptococcus pneumoniae, Streptococcus pyogenes and Haemophilus influenzae are the most Irequent.
13
is a fairly uniform 'grey hepatisation' of the teft lower lobe with five small foci of consolidation in the upper lobe adjacent to the oblique fissure. This appearance is due to the massive influx of inflammatory cells, associated with relative ischaemia. Com plications of lobar pneumonia include the development of septicaemia, an empyema, a lung abscess or carnifi cation (extensive fibrosis ).
Flg.2.5 Staphylococcal pneumonia. A close ''I' VII ,W " I
t:hows numerous characteristic foci of centriloblll; \I ' .111 '1" I which. in the upper centre, have coalesced to fo""., """ II abscess. Staphylococcal pneumonia most 011 01 1 (; 1JI"l llh ,. Infections or is nosocomial in origin. Suppuratioll lllil l \II Ifl ' ntion are simitarly seen in Klebsiella pneumonia, wli ll Ilospital-acquired infection. Both carry a relalivolv IIIIII! III" Irlt hose who survive , extensive lung damage IllftV l. tl ll.llI I
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2 Respiratory System Fig.2.3 Lobar pneumonia. Uniform red, firm consolidation of thi s left upper lobe, with comp lete sparin g of the lower lobe, is typical of 'red hepatisation' - the second stage of lobar pneumonia. This occu rs at about the 2nd to 4th days in an untreated patient , being preceded by engorgement and succeeded by 'grey hepati sation' and resolution at about the 8th to 10th day. Lobar pneumonia, particularly in its classical form, is rarely seen nowadays with the advent of modern an tibiotic therapy. How ever, young ad ults are most often affected, over 90% of cases being due to Streptococcus pneumoniae.
Fig.2.4 Lobar pneumonia. There is a fairl y uniform 'g re y hepatisation' of the left lower lobe with five small foci of conso lidation in the upper lobe adjacent to the oblique fissure. Thi s appearance is due to the massive in flu x of inflammatory cells, associated with relative ischaemia. Com plications of lobar pneumonia include the development of septicaemia, an empyema, a lung abscess or ca rnifi cation (extensive fibrosis).
F1g.2.5 Staphylococcal pneumonia. A close·up view of this lung ·;hows numerous characteristic foci of centrilobular suppuration wh ich. in the upper centre, have coalesced to form a small pulmonary nbscess. Staphylococcal pneumonia most often complicate s vira l IIllection s or is nosocomial in ori gin. Suppuration and abscess form It ion are similarly seen in Klebsie lla pneumonia, which may also be a Ilospital-acquired infection. Both carry a relatively high mortalily and , II I those who sUNive, extensive lung damage may remain. Fig.2.6 Aspiration pneumonia. The apex of this lower lobe shows multiple small foci of pale consolidation with microabscess form ation principall y locali sed around the small er airways. Aspiration pneumonia follows inhalation of material from the oropharynx, oesophagus or stomach and is commonest in un conscious patients, alcoholics and those with an upper alimen tary obstruct ive lesion . Aspiration of sterile gastric secretions is known as Mendelson's syndrome .
Fig.2.7 Lipid pneumonia. This lung shows uniform pale, rather waxy, consolidation . Note also the pre-existent bronchiectasis and centriacinar emphysema . Lipid pneumonia may either be exogenous,due to inhalation of ingested or regurgi tated oils taken in medication or food , or may be endo genous, occurring most oft en distal to an obstructing bronchial carcinoma and result ing from excessive accummulation of surfactant .
Fig.2.8 Lung abscess. This lung has been hemisected to show a large necrotic abscess cavity in the upper lobe. Note also the marked congestion and pre-existent bronchiectasis. Lung abscess is most often due to infection by Staph ylococci, Klebsie lla or Pneumococcus Type 3. It may develop after inhalation of foreign material or result from a septic embolus . Such abscesses are commonest in the upper lobes and may be complicated by rupture into a bronchus or the pleural cav ity, pleurisy or extensive lung scarring . Alternative ly , they may become wa lled-off and resolve .
14
2 Respiratory System
CAUSES OF BRONCHIECTASIS Mucoviscidosis CONGENITAL Bronchial malformation
Pulmonary infection
unresolved bronchitis or pneumonia viral lung infections tumour
ACQUIRED
Bronchial obstruction
hilar lymph nodes (especially TB) foreign body
Fig.2.9 Causes of bronchiectasis. Fig.2.10 Bronchiectasis. There is marked dilatation of most of the bronchial tree. Many of the bronChi contain purulent material and extensive broncho pneumonia is present. Bronchi ectasis is defined as irreversible dilatation of the bronchi associ ated with chronic suppurative infection . The lower lobes are most often affected and areas of dilatation may assume a fusiform or saccular appearance . Comp lications include lung abscess, empyema, lung damage (leading to 'cor pulmonale'), infective endocarditis, mycotic aneurysms and secondary amyloidosis .
15
Fig.2.11 Primary tuberculosis. Just beneath the pleural surface (lel1) is a small, pale nodule (Ghon focus) . the hilar lymph nodes show fibrosis and calcification The combination of these two lesions is known as a primary complex, which in this case is resol ving . Primary pulmonary tuberculosis remains endemic in underdeveloped countries and is almost always caused by Mycobacterium tuberculosis. Children or young adults are most often affected . Most lesions heal spontaneously, but progressive infection with abscess formation, bronchopneumonia or miliary spread may occur. Fig.2.12 Post-primary tuberculosis. In the apex of this lower lobe, there is an irregular cavity, containing caseous material, which has ruptured into a bronchus resulting in intra pulmonary bronchopneumonic spread . Post-primary tuber culosis is far more often due to exogenous reinfection than reactivation of previous endo genous infection. The lobar apices are typicall y affected and other complications of cavitation include spread to the upper res piratory or alimentary tracts. Secondary amytoidosis may develop in long-standing cases .
Flg.2.13 Miliary tuberculosis. Throughout ti,,' II"" I I i,Ii ,'"\nd particularly numerous around blood ve s~,d' " "I" ',1" 'tubercles' Mitiary spread is due to haemato<j" i!III1· , . I, " , Mycobacteria and may complicate either prlln:IIY 1111", III estructive foci erode into blood vessels) or i'('< I(.II Vdtl li I I I1l lection in debilitated, elderly patients. rill ·;> 1'1
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2 Respiratory s~eJ
2 Respiratory S) .. Inll
Fig.2.13 Miliary tuberculosis. Throughout the lung parenchyma, and particularly numerous around blood vessels, are small discrete 'tubercles' Miliary spread is due to haematogenous dissemination of Mycobacteria and may complicate either primary infection (in which destructive foci erode into blood vessels) or reactivated post· primary Infection in debilitated, elderly patients.
pUlmonaryl!'P" I
17
Fig.2.14 Tuber culous broncho pneumonia. The right lung shows multiple foci of caseous pneumonia. At the apex of the left lung a small subpleural area of scarring and caseation is apparent (arrowed) Tuberculous bronchopneumonia typically compli cates post-primary (reinfection) disease and results from the intrabronchial spread of the lique fied contents of a caseous cavity.
Fig.2.15 Cavitating tuberculosis. T III ~, I '. an upper 10bectQIIIY specimen whlc ll contains a rag ged, haemorrhagic cRvlly extending just beneath the visco!': II pleura The cavity I:, surrounded by arl area of pale caseolr :. necrosis. Such an appearance may represent pro gressive primary t JI. more common ly, post·primary infection and result:, from liquefaction Il f caseous matenal
Fig.2.16 Pulmonary embolism. The right main pulmonary artery is virtually occluded by a massive laminated thrombus. The lung I', rather pale in appearance Pul monary embolism most of tell complicates deep venous thromboses in the lower limb (see Fig. 141) The commo n()~ 1 predisposing factors are pro longed bed rest, particularly after surgical operations, partull tion, congestive cardiac failure and hypercoagulability. Mas$ lV<' emboli, such as the one showli here, prevent the passage of blood into the pulmonary cirelll ;1 tion and result in sudden d ea tll Smaller emboli, which lodge III more distal vessels , may havt, III ' effect, or may result in pul· monary hypertension and call>'" infarction (see Fig. 2.17) or m:ty give rise to pulmonary haemosiderosis.
Ie.
2 Respiratory System Fig.2.17 Pulmonary infarct. At the tip of the lower lobe is a wedge-shaped area of typical, dark-red infarction . Proximally, two branches of the pulmonary artery are occluded by embolic thrombus . Pulmonary infarcts are commonest in late adulthood and are predominantly a comp lication of deep venous throm bosis (see Fig . 1.41). Most pulmonary infarcts are of the 'red' congested type as true ischaemic necrosis is prevented by the dual blood supply from the bronchial artery.
1-_-
Fig.2.19 Coal workers' pneumoconiosis. The lung parenchyma shows patchy dense anthracotic pigmentation, a pattern known as dust reticu lation. Note also the character· istic mild centrilobular 'focal dust' emphysema. In addition a small, black, sil icotic nodule is present (arrowed). Simple dust reticu lation results from long term exposure to coal dust ; the development of nodules is due to co-inhalation of silica . There is no increased risk of lung cancer .
Fig.2.21 SlIIco. l.
I
parenchY"la It l 1111111,,, shows den~;u 111111 11 11' , mentation and 11111111111 culous cavity ill II " , III U hilar nodes ur(l 1.1111 I, I enlarged and tl ll l lll" ,, 1 fissure is SCUll lid ' :,11' seen most olion II I 11 1111 workers in tho '.111111) ,.1 industries. L I III\ I dllll i actually re!';lIit(l lllllllll i of macrOpllO\I'· ( "II, 'j lowing silicCl iltlh .. 111 11 and is morp. 1I:.'iI Illy, Ii ised by nOUlllor 11111, 1111 Fig .2.22). TubUl' 1I1i ,,~ 1 commoncorrll,l" ,111111
CA_USES OF PULMONARY HAEMOSIDEROSIS chronic left ventricular failure
Pulmonary hypertension
mitral valve disease left atrial myxoma
Goodpasture's syndrome Long-standing haemochromatosis Haemosiderosis FIg.2,18 Causes of pulmonary haemoslderosis.
17
Fig.2.20 Progressive massive fibrosis. This coal miner's lung shows, in addition to dust reticulation , large, well demarcated , black fibrous masses and smaller black nodules (top) . Progressive massive fibrosis affects up to 1"10 of coal miners and may also be seen in silicosis. The precise pathogenesis is unknown but it is thought that the degree of dust exposure and the possible coexistence of tuber culosis are important factors . The smaller nodules seen here are probably silicotic in nature , since coal dust often has a high silica content.
Fig.2.22 Haematlte pneumoconiosis. The IUIIU Pi li' " "
shows severe brick-red pigmentation with evictor 11 :11 1,1 111 I diffuse fibrosis and emphysematous change. nlhl !' 1I11 1il . inhalation of iron oxide, is seen most often in irOIl Ill,·,"II1' development of fibrotic lesions is again dependlll it 111 1111 . I existence of silica in the inhaled dust. Well recoqlll' I'H Ii, include tuberculosis and bronchial carcinoma.
2 Respiratory System Fig.2.21 Silicosis. The lung
Flg.2.19 Coal workers' pneumoconiosis. The lung
parenchyma is markedly fibrotic, shows dense anthracotic pig mentation and contains a tuber culous cavity in the apex. The hilar nodes are black and enlarged and the interlobar fissure is scarred . Silicos is is seen most often in miners and workers in the stone and glass industries. Lung damage actually results from the re lease of macrophage cell contents fol lowing silica-i nduced cell death and is more usually character ised by nodular fibrosis (see Fig .2.22) . Tuberculosis is a very common complication .
parenchyma shows patchy dense anthracotic pigmentation, a pattern known as dust reticu lation . Note also the character istic mild centri lobular 'focal dust ' emphysema. In addition a small, black, silicotic nodule is present (arrowed) . Simple dust reticulation results from long term exposure to coal dust; the development of nodules is due to co-inhalation of si lica. There is no increased risk of lung cancer .
L
CAUSES
O~ HONEYCOMB LUNG_ _ _
---..J
Pneumoconiosis
I I
Extrinsic allergic alveolitis
I
Cryptogenic fibrosing alveolitis (Hamman-Rich syndrome)
I I
Sarcoidosis Drugs/irradiation
I
Rheumatoid disease Systemic sclerosis Extensive pneumoniafTB Pulmonary eosinophilia Fig.2.23 Causes of honeycomb lung. Fig.2.24 Honey comb lung. The
,II\.II •••'lIn mnaalve fibrosis. This coal miner's lun g 1, 11111,1, 1.,1 Ii 11 ,1 II :Ik;ulalion, large, well demarcated, black 11111 "Iu lll' li 1.1lack nodules (top). Progressi ve massive ,I 'I i I'" I· ·.. ( ,I I :')01miners and may also be seen in " 1'1' • I·' plllhll(lollesis is unknown but it is thought that ,I . Ii or,1 '''' IIII~. l lfj I .lnd the possible coexistence of tuber III/ " ,,/ 11,1 101, I, 0/', I lie smalle r nodules seen here are " • III' III '011/, "" . 1,1I":e coal dust often has a high silica
.2.22 Haematlte pneumoconiosis. The lung parenchyma
I",w!: severe brick-red pigmentation with evidence of nodular and
• IIIII I1iU fibrosis and emphysematous change . Th is condition, due to 1I11 1f11HliQn of iron oxide, is seen most often in iron ore miners . The , I, 'V I ,to .oment of fibrotic lesions is again dependent upon the co 1I_I'.l ollce of silica in the inhaled dust. Well recognised complications ,to "I" tubercu losis and bronchia l carcinoma.
I".
apex of the lung contains numerous variably-sized cystic spaces, each having a thick fibrous wall. These cysts represent gross d i latation of bronch ioles and small bronchi in com pensation for destruction and fibrosis of neigh bouring alveoli and respiratory bronch ioles. Th is appearance re presents the end stage of va rious disease processes, the commonest of which are listed in Fig . 2.23 .
l~
2 Respiratory System
LUNG ACINUS
D
o o o
terminal bronchiole respiratory bronchiole alveolar duct alveolus
Fig.2.27 Centriacinar emphysema. In the lung parenchyma, small dilated air spaces surrounded by black anthracotic pigment are visible at the centre of the lung lobules . The surrounding alveoli are spared . These spaces correspond to the respiratory bronchioles and this is the commonest variant of emphysema, seen predominantly in cigarette smokers (especially males) . The upper lobes, particularly the apices, are most often aHected. A similar appearance is seen in coal workers (focal dust emphysema) in which there is usually little fibrosis or destruction .
Fig.2.25 A lung acinus. 3-5 pulmonary acini constitute a lung lobule.
CLASSIFICATION OF EMPHYSEMA Centriacinar Focal dust (in pneumoconiosis) Panacinar Paraseptal (bullous) Irregular Surgical (interstitial) Fig.2 .26 Classification of emphysema. With the exception of surgical emphysema, any lung may commonly show a mixed pattern of involvement.
19
Fig.2.28 Panacinar emphysema. In this lung note the much larger, confluent, dilated air spaces replacing complete lung acini. In places there is also a cent riacinar component. Panacinar emphysema , which is also very . common, affects the air spaces, including alveoli, distal to the terminal bronch ioles. In its classical form it is associated with Ct,-antitrypsin deficiency and previous bronchial obstruction . Most often, the lower lobes, particularly the lung bases, are affected.
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Fig.2.30 Pulmonnry hamartoma .. 1t1(.II IIIIII pleural surfac " 1111111" t. is a very well iI.11 pale tumour. I h(l " ,",,,11 the lung is I1UII 11111 1'11111 hamartomas W' l l lIlt III" developmenl nl l ll llllllllil usually cartil aOH H" ", II , which are only 1111 " Iy 'IV matico They ,UCiIYI III 11 11 pleural in 10c;tll",I. 1111; 1' more than feIl1l,1I ;- ., .1111 entirely beniql1
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2 Respiratory System
:."Irlllclnar emphysema. In the lung parenchyma , small ' I " I, n· . . "II rounded by black anthracotic pigment are II" \ ' ·,,1,,· ot the lung lobules . The surrounding alveoli are II I, "" .. 'I ql COS correspond to the respiratory bronchioles and i ,ti l" 11( 11 H >f;t variant of emphysema. seen predominantly in II" d·. II ', ( ..'specially males) The upper lobes. particularly , II" "" 1:,1 Ollen affected . A similar appearance is seen in " I (I '" [I I dust emphysema) in which there is usually little I
11
I
4
I. " .1~ ll~ ,I'IC)I I .
Fig.2.28 Pan acinar emphysema. In this lung note the much larger, confluent, dilated air spaces replacing complete lung acini . In places there is also a cent riacinar component. Panacinar emphysema, which is also very . common, affects the air spaces. including alveoli, distal to the terminal bronch ioles. In its classical form it is associated with ai -antitrypsin deficiency and previous bronchial obstruction . Most often, the lower lobes, particularly the lung bases, are affected
Fig.2.29 Paraseptal emphysema. At the apex of this lung is a large emphyse matous bulla with a fibrous wall. The adjacent paren chyma shows mixed centri- and pan acinar change . Paraseptal emphysema predominantly affects the alveoli adjacent to the inter lobular septa or pleural surface . It is usually most pro nouncedinthe upper lobes, often close to an area of previous scarring. This variant is the usual precursor of bullous emphysema and is often seen associated with other variants, as in this case .
Fig.2.30 Pulmonary hamartoma. Just beneath the pleural surface of this lower lobe is a very well demarcated, small . pale tumour. The remainder of the lung is normal. Pulmonary hamartomas are not uncommon developmental anomalies, usually cartilaginous in nature , which are only rarely sympto matic. They are typically sub pleural in location, affect males more than females. and are entirely benign.
Fig.2.31 Bronchial 'adenoma'. In the main bronchus is a smooth . well circumscribed tumour projecting from the epithelial surface . These lesions may be derived either from submucosal glands or neuro endocrine APUD cells and are misnamed since they represent low-grade, malignant tumours which may eventually metastasise . They most often arise in young adults and there is usually extension into the adjacent lung parenchyma. Fig.2.32 Hilar bronchial carcinoma. Arising from the lower lobe bronchus , close to the hilum. is a pale neoplasm which is irregularly infiltrating the parenchyma. Bronchial carcinoma most often originates near the hilum and may be squamous (50%), small cell (oat cell) anaplastic (20%), adeno-(15%) or large cell ana plastiC (10%) in type . It is the commonest cause of death from malig nancy in Great Britain and in many cases is associated with cigarette smoking or industrial exposure to carcinogens. The overall 5-year survival is only between 5 and 10% .
20
2 Respiratory System Fig.2.33 Bronchial carcinoma with distal bronchi ectasis and broncho pneumonia. At the apex of the left lower lobe is a partly necrotic. pale neoplasm which has obliterated the lower lobe bronchus : distally the smaller bronchi are grossly dilated (bn;Jnchi ectasis) and the remaining paren chyma shows consolidation . The adjacent middle lobe shows confluent broncho pneumonia. These are common comp lications of obstruc tive bronchial carcinoma and may also be accom panied by collapse or abscess formation . Fig.2.34 Peripheral lung carcinoma. Just beneath the pleura of the oblique interlobar fissure is an irregular, well demarcated, pale tumour which is situated well away from the main bronchial tree. The majority of peripheral primary pulmonary malignant tumours are adeno carcinomas which compri se about 10-15 % of all lung cancers. These tumours show an equal sex incidence and tend to arise in foci of scarring . An apparently slow growth rate'and frequent operability means that they carry a better prognosis than most bronchial carcinomas.
21
Fig.2.35 Bronchioalveolar carcinoma. The entire lung is diffusely infiltrated by a pale neoplasm which, particularly in the upper lobe, has adopted a nodular appearance. Bronchioalveolar carcinoma comprises about 2% of all primary lung cancers and is a specific variant of adenocarcinoma, which tends to spread extensively within the air passages. Its diffuse nature often prompts mistaken clinical diagnoses of an infective or inter stitial disorder.
Fig.2.36 Multiple pulmonary metastases. Beneath the pleura and in the lung parenchyma are innumerabte pale, umbilicated nodules of tumour. Up to a third of patients dying of malignant disease have pulmonary metastases, the commonest sources of which are carcinoma of the breast, colon, stomach and lung itself. The presence of an extensive vascular and lymphatic system in the lungs is responsible for the predilection that metastases show for this site.
C! Il lqtt t. 1I I
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Ig.2.38 Pulmonary lymphangitis carcinomato.n I I ,. ',lIdace of this lung shows innumerable small spl ,,,"',tl "' I iI 'posits of pale tumour. This represents extell:.;iv" II ,ldl" i I" ilrnonary lymphatic channels, which are fillec1I,y '''1,1, . IIHI may be caused by either primary or seconda,y "" 'tl \
I"
2 Respiratory System Fig.2.35 Bronchioalveolar carcinoma. The entire lung is diffusely infiltrated by a pale neoplasm which, particularly in the upper lobe, has adopted a nodular appearance. Bronchioalveolar carcinoma compr ises about 2% of all primary lung cancers and is a specific variant of adenocarcinoma, which tends to spread extensively within the air passages. Its diffuse nature otten prompts mistaken clinical diagnoses of an infective or inter stitial disorder .
Fig.2.37 'Cannon ball' pulmonary metastases. In thi s congested lung. fo ur well circumscribed , almost spherical, deposits of pale metastat ic tumour are present. This appearance of a small number of large secondary depos~s i nthelung.
while not entirely specific , is classic ally associated with spread from renal adenocarcinomas or testicular tumours.
Fig.2.39 Pleural hyaline plaques. On the parietal pleura of the posterior thoracic wal l are several foci of yellowish hyaline thickening . This appearance is most otten seen in individuals who have suffered prolonged exposure to asbestos, usual ly in the course of their occupation. Such patients also commonly develop macroscopically non-specific pulmonary fibrosis , collapse or bronchiectasis of the lower lobes. Crocidolite is pathogenetically the most dangerous type of asbestos and occasiona lly only very brief exposure is sufficient to induce pulmonary disease . Fig.2.40 Mesothelioma. Th is apical portion of the lung is encased in pale, infiltrative tumour arising from the pleura. Involvement of the soft tissues at the apex is also apparent. Malignant mesothelioma is uncommon and may arise from the parietal or visceral pleura . The vast proportion of cases arise in patients exposed to asbestos, usually occupation ally, and such individuals or their families are entitled to indust rial compensation . The prognosis is uniformly appalling .
1111"1(,, "ulmonary metastases. Beneath the pleura and
I IYI II.I nro innumerable pale , umbilicated nodules of ,11 11111 I>~ pat lonts dying of malignant disease have , \I Id',h","· .. tho commonest sources of which are .111 11 I )11,01·,1. colon , stomach and lung itself. The •., " ' \\ 'I" ;IV" vascular and lymphatic system in the lungs I, ., II,, · I ,,( '(hlnClion that metastases show for th is site.
\ " .. , II III
Ig.2.38 Pulmonary lymphangitis carclnomatosa. The pleural
, " Ir1 t i.l of this lung shows innumerable small spherical and li near
.'''111 i' ,il S of pale tumour This represents extensive infiltration of the
I" 11""" IGry lymphatic channels, which are filled by neopl astic ce ll s,
., " "" IIY be caused by either primary or secondary lung tumours.
22
f
j j d ,tit __
!
dII d
3 Alimentary System Fig. 3.1 Oral leukoplakia. This clinical photograph shows ex tensive smooth white patches over most of the tongue . Leuko plakia is purely a clinical des cription of any white plaque and is not a pathological diagnosis . In many cases, oral leukoplakia is benign, representing hyper keratosis, the commonest causes of whic h are chronic irritation or smoking. Only cases which also show epithelial dys plasia can be regarded as pre malignant. Other causes of white lesions in the oral cavity include lichen planus and candidiasis .
'I
'Ii
Fig.3.3 Mixed salivary tumour (pleomorphic adenoma). Thi s is the commonest neoplasm of salivary gland s, the parotid being the most frequently aHected site. Th is section shows a fairl y well circumscribed, multinodular tumour; the cut surface has a myxoid cartilaginous appearance and there are small foci of cystic change and haemorrhage . These tumours are prone to local rec urrence , mos t often as a consequence of spread th rough the capsule, which results in incomplete surgical excision . Malignant transformation is exceed ingly rare .
Fig.3.2 Squamous carcinoma of tongue. This clinical photograph shows an irregular, raised pale lesion on th e inferior surface of the patient's tongue. Most malignant tumours of the oral cavity are squamous carcinomas and postulated aetiological factors include tobacco smoking, syphi lis and drinking strong spirits. They most commonly present in late adulthood , aHecting predominantly males. The clinical course is very variable but carcinoma of the tongue generally carr ies a worse prognosis than tumours si tuated elsewhere in the mouth.
23
..
Fig. 3.5 O••oP'''' '' dldlasls.IIII1I1III I11I·1 oesophO\JlIiI iI.11I11 11I immunOCCllllpl' " lli· d whether Ihoy II" '1 " 1'I tated (parlrcilli rrly I I ~ I disease), rUt;tJIVil " 1' 1 chemothcrrlPY I" I U ri a primary il1l1l1l II I"" II order. Th e :,Ilvllllly " tion is deprH" 1,·, I( 111" degree of tJ"hlllly , .,, ' appearancI1 ", II ''' I may rango 11 (111 I 1111 ' ~ I plaque s Witl, llli"'"I,. i mation to UI OYI· II" III, lesions with fllII l1II II III forma tion niH t ,," ,1111> mation, (1:': ::111111 11111 '
Fig. 3.4 Pharyngeal pouch. The pharynx has been opened posteriorly to show a diverticulum extend ing laterally. A pharyngeal pouch is a pu lsion diver ticulum which occurs at Killian 's dehiscence, due to neuromuscular in coordination of the pharyngeal con stri ctor muscles. Elderly males are predominantly affected and ve ry occasionally post cricoid carcinoma may develop in sucll a pouch .
III J.6 Oesophagus - peptic (Barrett's) ulcer. II "' I,Iq [,I Ir tlil icer with a haemorrhagic base is presollt III "," " 'I II" ' Io;:,ophagus . Barrett's ulcer occurs as a cOIII"Ii,
3 Alimentary System Fig. 3.5 Oesophageal can didiasis. Fungal infection of the oesophagus is not uncommon in immunocompromised patients, whether they be generally debili tated (particularly by malignant disease), receiving cytotoxic chemotherapy or suffering from a primary immunological dis order. The severity of the infec tion is dependent upon the degree of debility, and the appearance in the oesophagus may range from flat white plaques with minimal inflam mation to greyish, ulcerated lesions with pseudomembrane formation and florid inflam mation , as seen here.
...1I ••lIvory tumour (pleomorphic adenoma). Th is is ,. I , " 11 '1 )111' ,111 of salivary glands, the parotid being the ' 1I 1V 1111nl.llld site, This section shows a fairly well " HI, I""Ilill(')(\u lar tumour; the cut surface has a myxoid
Ii 'I ,, '," , II Ice and there are small foci of cystic change 11 1()ne tumours are prone to local recurrence, II , ' I 1I11' .'lquence of spread through the capsule, which , ," '1,1, ,t, I curg ical excision. Malignant transformation is illdlf "
, IIIH
Fig. 3.4 Pharyngeal pouch. The pharynx has been opened posteriorly to show a diverticulum extend ing laterally. A pharyngeal pouch is a pulsion diver ticulum which occurs at Killian's dehiscence, due to neuromuscular in coordination of the pharyngeal con strictor muscles. Elderly males are predominantly affected and very occasionally post cricoid carcinoma may develop in such a pouch .
'i
1tllllll,hagu8 - peptic (Barrett's) ulcer. A sharply demar
, II" ' I I,IIul norrh agic base is present in the lower third of
I! 1\ I' '10 11111' il lI' ~ ulce r occurs as a complication of either
, Ii ,I "11 , ' 11 lililorotopia within the distal oesophagus. The
11101 "" Icllux oesophagitis, often in association
I
Fig. 3.7 Oesophageal stricture. The posterior aspect of the oesophagus has been displayed to show a zone of stricture formation complicated by the development of a small acute ulcer. Note the gross di latation of the proximal (upper) oesophagus . Oesophageal stric tures may be caused by a variety of conditions includ ing reflux oesoph a(Jitis, peptic ulceration, ingestion of corrosives, scleroderma or trauma. Clearly, it is essential to distin guish such benign lesions from a stenosing carcinoma. Fig, 3.8 Mallory Weiss tear. The Mallory-Weiss syndrome is an uncommon cause of haematemesis, in which, most often, violent or prolonged vomiting results in tearing of the oesophageal or fundal mucosa with damage to the underlying blood vessels. It is par ticularly common in alcoholics.
I "","I', I
24
3 Alimentary System Fig.3.9 Oesophageal varices. These are dilated veins, situated predominantly in the lower oesophagus, which develop as a complication of chronic portal hypertension, most often due to cirrhosis of the liver . They are prone to rupture with resultant haematemesis . The oesophagus has been opened long itudinally to displ ay numerous tortuous, dilated vei ns and. in the lower half of the picture, a mass of blood clot is present in the stomach .
Fig. 3.10 Achalasia. The oesophagus and gastric fundus have been opened to dis· play gross dilatation of the oesophageal lumen. The oesophago·gastric junction . not visible in this pi cture , wa s very narrow . In the posterior wall of the distal oesophagus are two pulsion dive rt icula. Achalasia is an id io palhic disorder of neuromuscular co ordination affecting the autonomic plexus in the distal oesophagus; the oesophago-ga'stric junction fails to relax during swallowing resulting in proximal dilatation .
25
Fig.3.11 Carcinoma of the oesophagus. The oesophagus has been opened longitudinally to show an exophytic. largely ulcerated carcinoma in the middle third . Squamous carG:inoma is the commonest malignant tumour of the oesophagus and most often affects older adults. predominantly males. Smoking and a high alcohol intake are thought 10 be causally related . Tumours in the upper third may rarely occur in association with the Plummer-Vinson syndrome which is almosl exclusively seen in females, Tumours in the distal third of the oesophagus are most often adenocarcinomas which arise either in area s of gastric metaplasia or heterotopia. or represent infiltration by an adjacent gastriC primary tumour .
Fig.3.12 Congenital pyloric stenosis. This infant's stomach has been opened to show marked hypertrophy of the muscle coat at the pylorus with obstruction and proximal dilatation . This condition is idiopathic but usually presents in the neonatal period with projectile vomiting , It occurs in approximately 1 in 500 live births . is common ly familial and affects males more than females . The mode of inherit ance appears to be multifactorial. If untreated. the patient may develo p pro found metabolic alkalosis.
Fig. 3.13 Acute gastritis. Th is stomach has be,," III "" normal rugal pattern with marked mucosal congO! ;I" "' " , " hand side (ef the pyloric antrum on the left) Acull' 11' 1' ,11 defined as transient mucosal inflammation, Ihe CUll whiCh are salicylates, excess alcohol intake. cyl'"II '~1I III hypotensive shock (of whatever cause), Added illl"IIII'" oedema and haemorrhage may lead to the devo" "I" ", "~ I mucosal erosions,
"''' '"I
I" Acute gastric ufcer. The stom Gtcl1l"", I""", , 'I 1'.1" 'flow, ,,'Inched-out haemorrhaO 'G III, HI w,II, • , I I' fI ' III" 1)( :pl ,e ulcers represelll all nXI"Il !iH " ' , " II' , , 1" , " 1/ "." "'S ano as sue r, have larqllly II,,· ' .,11 ,,(1 , I , I,ll,,,,,, 11':11, :(1 from an erosion I) y ""'11 lI'v, ,l vII"" , " ,', ,'.,', w,'lI:ls 111C mucosa. At.I,II : 'II""" .I , ""I ~ , 1,1~ ! j
, I
j
1 1t,.·.{ ;(Jri1lnOn {1~S OClil l( ~dr:;II I ~ ,(, : ,"t l ll ll ll l
fll, ',,)II" ;"ll:IlIill IIljlllY Hnrf ''''"111',11' '1(1'\1(( "
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3 Alimentary System
"' 1111 '"11' 01 the oesophagus. The oesophagus has I I, ,,,, 111 111 III',,II,V I( i :·,ll0Wan exophytic , largely ulcerated , II" . I il li h II" II III cI ~qua mous car€inoma is the "" ti l> 11 "1 11 1\lIIIUIH 0 1 the oesophagus and most ollen I, IIIIi'. Iii'" II IlIlWICl illly males, Smoking and a high , I" " II h.. " 1111 I!I 1)0 causall y related, Tumours in the ' i IY ' IIl'ly 111.(;11 1 In association with the Plummer·Vinson I", 1"" ,,111,11' ;1 i 'xctlJslvely seen in females Tumours in ,d , 011111" II ",Iii diOguS are most ollen adenocarcinomas 111,( " IL l, II", 11 , 0 1 gastric metaplasia or heterotopia, or 1,11, , \I " Ililly, ,, \ (Klpqcent gastric primary tumour.
""IIQIVIHI pyloric stenosis. This infant's stomach has , li d ',IIIW11I.III,I·d 11Vpertrophy of the muscle coat at the " ,1 1',111 ' 1I"Ii ,Hid pI<Jxi mal dilatation, This condition is 11 11" ',Il,,'iy I'" " ,.,"t', HllIlC~ neonatal period with projectile , ', ; , " ~ " " I " ," "' IIII:llr'ly 1 In 500 live births, is commonly I ,11" , 1'.",..1, .', 111""' 1I1,,"I(~lnal es, The mode of inherit· " • I, , 1'1",,,11,1.;, II If I, II 1IIIIIIreated , the patient may , ,II " "1\ I '" lldl 'I !II , itlll' ,1(,' ,I',
Fig.3.13 Acute gastritiS. This stomach has been opened to show a normal rugal pattern with marked mucosal congestion on Ihe right hand side (eI. Ihe p yloric antrum on the lett) . Acute gastrilis is defined as transient mucosal inflammation, the commonest causes of which are salicylates, excess alcohol intake, cytotoxic drugs or hypotensive shock (of whatever cause), Added intramucosal oe dema and haemorrhage may lead to the development of small muc osal erosions ,
Acute gastric ulcer. The stomach has been opened to 11,1111 IW, punched·out haemorrhagic ulcer with smooth 1\1 "1,, poptlC ulcers represent an extension of acute gastrilis I I I" , "" I' ,1!lII'" ;~nd as such have largely the same causes, They II I " , Iilil ,I' 'I 11r; Ilc'd from an erosion by their involvement of the ,111111." '" I d ' , WI 'II ,j ,; IIle mucosa , Acute ulcers rarely exceed 1 cm i J~ (l i " I. I I, ",', I Ilfllfllon associated causes include severe burns Ililfi. i "I " q) I'll 'I)ral injury and neurosurgery (Cushing's 'I I
Fig.3.15 Chronic gastric (peptic) ulcer. The stomach has been opened to show a sharply demarcated ulcer with straight edges, which has penetrated the muscular layer of the gastriC wall; its base is composed of smooth but irregularly heaped-up granulation and scar tissue , Chronic gastric ulcers affect males more than females, usually in middle life and are more common in patients with blood group 0, Most patients are usually hypochlorhydric and it is thought that relative mucosal ischaemia, an impaired mucosal mucous barrier, altered gastric emptying rate or reflux of bile acids from the duodenum may be important pathogenelic faclors , These ulcers are usually solitary and the vast proportion arise at the border zone between acid-secreting and non-acid-secreting mucosa, particularly on the lesser curve, Fig.3.16 Bleeding gastric ulcer. Chronic peptic ulcers commonly damage small arteries or veins as Ihey erode the stomach wall, Haemorrhage is Iherelore the most Irequenl complication : this may either occur in small amounts over a long period 01 lime, resulting in melaena and iron-deficiency anaemia , or a larger vessel may bleed acutely and heavily, giving rise to haematemesis , There is clotted blood in the Iloor of this ulcer and blood in Ihe stomach, Nole also Ihe smaller peptic ulcer jusl above Ihe main lesion,
tI' (
26
3 Alimentary System
Fig.3.17 Perforated gastric ulcer. Perforation of chroni c peptic ulcers. i.e. disruption of the full thickness of the stomach wall, occurs in up to 5% of cases. This is a life-threatening complication which results in peritonitis . In this specimen, while a small area of granu lation tissue remains at the superior border of the ulcer, perforation has oc curred and part of the left lobe of the liver is visible through the floor of the lesion.
Fig.3.18 Chronic atrophic gastritis. This'stomach shows extreme atrophy and pallor with loss of the mucosal folds and marked attenua tion of the gastric wall, such that it is almost translucent. This re presents the end-stage of chronic autoimmune gastritis, which i's the commonest cause of pernicious anaemia. Autoantibodies to intrinsic factor and gastric parietal cells are found in such patients, who develop a marked deficiency of vitamin 8'2' Up to 10% of patients with atrophic gastritis may subsequently develop gastric carcinoma.
27
Fig.3.19 Gastric adenoma. At the edge of the greater curve is a small, rounded, raised lesion projec ting from the mucosal surface (arrowed) . There is no evidence of ulceration or adjac ent infiltration . Gastric adenomas, more accurately known as neoplastic polyps, may be classified like those in the large bowel (see Figs .3.45 and 3.46) although they are only rarely pedunculated. They have exactly the same malignant potential and are often seen in association with chronic atrophic gastritis.
Fig.3.20 Gastric adenocarcinoma. In the fundus of the stomach is an ulcerated neoplasm with irregular rolled edges . Adenocarcinoma of the stomach is one of the most common causes of death due to malignancy in Britain, occurring most often in elderly men. There is a familial incidence and patients with blood group A are at increased risk. Geographically, the disease is most common in Japan and Scandinavia. Currentl y fa voured aetiologic al agents are nitros amines, derived from ingested nitrates which are used in preserva tives and crop fertilisers. Known predisposing conditions include chronic atrophic gastritis and uncommonly, gastric adenomata. Macroscopically, ulcerating tumours are far more common than the fungating or polypoid forms.
Fig. 3.21 Linitis plastica ('leather-bottle' stomaoh), has been dissected to show diffuse infiltration of II JlII I " curve by pale, rigid tumour, resulting in shrinka( JI' 01" " lumen . This macroscopic variant of adenocarcill('" Ii1 " I represents widespread infiltration by poorly diflol,\l lI lIlt. an associated dense fibrous (desmoplastic) stl'on ll l ~ " r tumours only rarely impinge on the gastric lumerl, present at an advanced stage and the prognosi:; n, V'" ,
"'"Y '
Ig.3.22 Gastric leiomyoma. The stoma cl) Ilw: I li")i1 III 'w I ' smooth, rounded and well circurn scrIhud III" " ,i I Itil I" 01its apex. The cut surface (right) revealn IIt.11 "" I I , " V, 111)( 1 by a layer of attenuated, normal OpilhlJllIII" I , II, I I' II II Ilr:ornmon benign gastric tumours, whiCI I ,"" , ,11 ,," II " rhey arise within the muscular coal (111111 1' ,II 1111 '1< '" 11I[)loc ting into the gastric lumen, am VllIY 1"')111111 ,.1> i '1.'lh ,n. Their malignant counterparl, loiolllY, I'"", 111 11 I'"I' I/ lly I,II U
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3 Alimentary System
'I
It IlliuJ1om a. Al lhe edge of the greater cu rve is a I ,,11.,,11 1It " 11011 rrojecting from the mucosal su rface ' Ii I' '"', 'VI( lonce of ulceration or adjacent infiltration . 'I I" I II" 11" .lccurately known as neoplastic polyps, lilt '1 1111,,, litora, in the large bowel (see Figs .3.45 and I, \1 11 ,.,. .11 '" 'Illy rarely pedunculated. Th ey have exactly 1I I' " " II 1"111 illi llil and are often seen in associalion with I II, 'I'" 11111 '.
hll ndl)nocarcinoma. In the fundus of the stomac h is ' llilll'dil willi Irregu lar rolled edges. Adenocarcinoma 1I II "11111111110 most common causes of death due to I. I 1111, "11 , "''1 111 ring most often in elderly men . Th ere is a , ' " .. ,11 1<\ " ,11 ;\\1115 with blood group A are at inc reased , .1,1, :,dl y, IIl!Jouse is most common in Japan and ! 1II" ,"l1y Il lv()lIred aetiological agents are nitros , " li lt 11 11 " il l" ',h lc! nitrates which are used in preserva '1, lf"I IIi ,.III'1 1\l IOWIl predisposing conditions include 1,111' 1/11' 11111' . IIlId IIlIc ommonly , gastric adenomata . IlIv "I, ,II , Ii II 10 111I11I"lr$ are far more common than the , " ,1 " 1" ,1.1 1111" II.
Fig. 3.21 Linitis plastica ('leather-bottle' stomach). The stomach has been dissected to show diffuse infiltration of muc h of the greater curve by pale, rigid tu mour, resultin g in shrinkage of the gastriC lumen . This macroscopic variant of adenocarcinoma of the stomach represents widespread infi ltration by poorly differentiated tumour with an associated dense fibrous (desmoplastic) stroma. Since these tumours only rarely impinge on the gastric lumen, they common ly present at an advanced stage and the prognosis is very poor
Fig.3.23 Chronic duodenal ulcer. The stomach and proximal duodenum have been opened to show a well circumscribed , deep ulcer with smooth edges in the first part of the duodenum . Duodenal peptic ulcers are much commoner than their gastric counte rpart s and are seen most frequently in males between the ages of 20 and 40. In contrast to gastric ulce rs, these lesions are associated with marked hyperacidity, the precise cause of which is uncerta in. They occur most often in the first pari, particularly on the anteri or wall and produce similar complications to ulcers in the stomach .
II ,
(I" .
3.22 Gastric leiomyoma. The stomach has been opened to ,IIIIW .' ~mooth, rounded and well circumscr ibed tumour , with a small Pi< I I dill:.; apex. The cut surface (right) reveals that the tumou r is "" ' " 11( IIJY a layer of attenuated , normal epithelium . Leiomyomas are ,,, ,11 11" 1IIIIIYlOn benign gastric tu mours, whic h are often asympI, ,". III' Ihoy arise within the musc ular coat of the stomach and, III II I '" '1 1lt:ling into the gastric lumen , are very prone to superficial
II, , I ""111 litei r malig nant counterpart, leiomyosarcoma, is ex-
Itt· ; 111 dy
/.1/1
I
Fig.3.24 Periampullary carcinoma. Thi s segment of duodenum has been opened to show a fungating, focally ulcerated tumour arising around the ampulla of Vate r. The pyloric canal is apparent on the right. Periampullary carcinoma ari ses from the distal end of the common bile duct and occurs most often in older adults. It is usually slow-growing and carries a relatively good prognosis. It is very important to distinguish such cases from carcinoma 01 the head of the pancreas , which pre sents very similarly, since surgical interven tion is undoubtedly worthwhile in periampullary tumours
28
) Al imentary Syste m
Fig.3.25 Meckel 's diverticulum. This opened segment of iteum shows a wide diverticutum . about 2 cm in diameter. lined by rather smooth mucosa. Meckel's di verticutum is a congenital malformation representing a remnant of the vitello-intestinat duct. Usually found about 60 cm from the ileocaecat valve, it affects about 2% of the poputation. White it may become inflamed or obstructed, ectopic gastr ic mucosa is present in some ca ses, which may tead to peptic ulc eration . Other ectop ic epithelia whi ch are often found include pancreatic , duodenal and cotonic types.
Fig.3.26 Crohn's disease. This opened length of small bowel shows the typical 'cobbtestone' appearance of the mucosa, each nodule being separated by ulcerated fissures . Crahn's disease is an idiopathic granulomatous condition which may affecl any site in the alimentary tract but shows a predilection for the terminat iteum . It presents most often in the 2nd to 4th decades. Postulated aetio logical agents include various micro-organisms and fine particutate matter, which have induced an abnormal immunotogical respon se. Multifocal invotvement , giving rise to 'skip' lesions , is characterist ic and inflammation of the full thi ckness of the bowel wall causes deep fissuring , fistula formation and fibros is.
29
Fig . 3.27 Crohn 's d isease. This opened segment of large bowel shows two quite separate 'skip ' lesions, characterised by florid mucosat ulceration . The tesion on the teft has induced marked luminal stenosis with obviou s proximat dilatation . Up to 15 % of patients with Crohn 's disease show large bowel involvement, with or without small intestinat d isease . There is a definite increased risk of colonic adenocarcinoma, but th is is much less marked than in ulcera tive cotitis .
Fig. 3.28 Typhoid ulceration . These segments of small intes tine have been opened to show several ovoid ulcers lying parallel to the bowel wall (cf . Fig . 329) The ulceration has oc curred al the site of necrosis of Peyer's patches. Typhoid fe ver remains endemi c in some parts of the world, especi ally As ia and the Far East. It is due to ingestion of food or drink con taminated with Salmonella typhi, usually from an asymptomatic carner. Important local com plications include perforation and haemorrhage. Following invasion of the bloodstream , excretion of Salmonellae in bile may tead to chronic gallbladder infection (whence the c arrier state) .
Fig. 3.29 Intestinal tuberculosis. In contrast tn I III I ulceration, while still originating in Peyer 's patcil t!t. . ,,, 10 versely around the bowel wall following the lines oltV" '1 I age . Intestinal tuberculosis may be primary, ret.llilll lIll " of unpasteurised milk, or secondary, as a con soq ll'" rr , ing infected sputum from pulmonary d isease . I\UI.II 111 ,1 I nodes are usually involved and may later undeIOP I IY'.II' calcification . Peritoneal involvement may lead to 11',( Ilf"
III 3.30 Small intestinal ischaemia. This 1001J I II 1!l IW 11111 1IIIIrkedly congested This is the apreArli1 II " ",I Uti li'
IIIIW," wall, but lesser degrees of ischacllliu III, IY I' " ,ri ll I J' ,dl llice ration . It most commonly resllil :, III " " .111 ",I' 1i1 ,.oIly ,,,Irrhac in origin, occluding il hmlJl:hlll II,,, '''II'' I III ,OII"IV Olher ca uses include Severn hy""I"I II,JI II' II, II I 1i1" "I' III 1;,touS vessel, retrograci e IIlfarc' tllll J
3 Alimentary System
IIhll' . tll.OBBe. This opened segment of large bowel HI' I ,1'1" 11.IItJ '!;ikip' lesions. characterised by florid I 11 11 11 1 II,,· I U I~ l on on the lett has induced marked , 1', wil l, " l lVl""S proximal dilatation Up to 15% of I "itll" '. I it!••;;lse show large bowel involvement. with or IIl dl ll !lI",I , iI·,ease. There is a definite increased risk of " I I'. 11 11 illl.,. ljllt this is much less marked than in ulcera
Fig.3.28 Typhoid ulceration. These segments of small intes tine have been opened to show several ovoid ulcers lying parallel to the bowel wall (cf. Fig . 329) . The ulceration has occurred at the site of necrosis of Peyer's patches Typhoid fever remains endemic in some parts of the world. especially Asia and the Far East. It is due to ingestion of food or drink con taminated with Salmonella typhi. usually from an asymptomatic carrier. Important loca l com plications include perforation and haemorrhage. Following invasion of the bloodstream. excretion of Salmonellae in bile may lead to ch ronic gallbladder infection (whence Ihe carrier slate)
Fig. 3.31 Mesenteric embolism. The superior mesenteric artery is totally occluded by thrombus which has emboli sed from the lett atrium in this patient with atrial fibrillation. Proximal occlusion. such as thi s, results in infarction of almost the entire small bowel and is invariably fatal Fig.3.29 Intestinal tuberculosis. In contrast to Fig . 3.28. this ulceration. while still originating in Peye r's patches. extends trans versely around the bowel wall following the li nes of lymphatic drain age . Intestinal tuberculosis may be primary. re sulti ng from ingestion of unpasteurised milk. or secondary. as a consequence of swallow ing infected spu tum from pulmonary disease. Adjacent lymph nodes are usually involved and may later undergo dystrophic · ~ Icification . Peritoneal involvement may lead to ascites.
3.30 Small Intestinal ischaemia. This loop of bowel is dilated 1I 11 111 11 111\C 'llly congested. This is the appearance of infarction of the I " ,w,,1 w.t ll, ill II lesser degrees of ischaemia may result only in II! II ''' lI ilIIIi .lllAlion. II most commonly results from an embolus.
I,' I, Ill y •."Ii h.ll; III o ri ~ lin , occluding a branch of the superior mesent-
I I, 11 1. 111/ I HI II !I Ciluses include severe hypotension. thrombosis in
III 11111 ' I'"l"h ll l( . v,,~~ se l , retrograde infarction due to mesenteric
" . •11 ' II I II 'II Ii J(/~ i l: i c)r diqltali s therapy .
Fig.3.32 Carcinoid tumour. The terminal ileum and caecum are shown here. Originating in the ileocaecal valve is a well circumscribed, yellow tumour in the submucosa. In the adjacent mesenteric fat, a lymph node containing metastatic tumour can be seen (arrowed) . Carcinoid tumours arise from neuroendocrine APUD cells and are usually found in the appendix or small intestine. Tumours in the appendix tend to be solitary and affect young adults, while those in the small bowel may be multiple and usually present in old people . The appendiceat neoplasms almost neve r metastasise, but small bowel tumours frequently spread to lymph nodes and the liver.
30
3 Alimentary System
-...-,. . ' t:.(,~.~~ " .. .~. . . ":":;"68~ \.1.,'. ....~t~',·I tl.~;; ,'.""'~~; , ~\,,", .':,',. '1 J'" }!~, .. ~ .~;'''' , to- ,,:\"" , , '.,!
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Fig.3.33 Intestinal lymphoma. This segment of bowel shows a solitary, ulcerated turmour Macroscopically, primary gastro· intestinal lymphoma may be indistinguishabte from a carcinoma . Involvement by disseminated lymphoma usually gives rise to multiple lesions and may therefore be more easily recognised . Gastro intestinal lymphomas are nearly all non-Hodgkin's in type . There is an increased incidence associated with coeliac disease and Ct heavy chain disease .
Fig. 3.34 Acute appendicitis. The appendix below shows marked serosal congestion while the one above is covered in a fibrino purulent exudate, indicative of more advanced infection . The aetio logy of acute appen dicitis is still debated but it is thought that luminal OIISIIIIClion, usually hy faecal malerial, probably leads to mucosa l Illcornliol1lollnwoei hy I'llllt~ l r:llin l l ()f tilt! I1IJwei wall hy:l wHioly of 11 '01;1\10101111,:",,(; (), : UI!lH 11 1111 I'll :rlili m; : ;\11 1II, ;t;i H In drflJlly III VIl! Y Yljlll l\l p" I" 11 11 ', wi", I II w
Fig.3.35 Acute appendicitis. This appendix has been sectionea transversely to show copious intraluminal and intramural purulent material associated with congestion and haemorrhage in the wall and adjacent mesenteric fal. Fig. 3.36 Ulcerative colitis. The distal portion of this rectum has a granular, almost velvety, appearance with haemorrhage and innumerable shallow ulcers. The proximal margin (above) appears normal. Ulcerative colitis is an idio pathic disease, pre dominantly of young adults, which always involves the rectum and affects the proximal large bowel in continuity. It is a chronic relapsing condition primarily affecting the mucosa. It is assoc iated with HLA R '? 7 and may Il() corn p l,c al ori l}y to ,xi.: II l1 l ll l ll:01(1I1.1'01
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Fig.3.37 Pseudo-polyps in ulcerative colitis. rlli" 14, large bowel shows intense mucosal congestion ;II,d, II I mucosa is 'thrown up' into innumerable irregulal I" ,IYI'" trusions . These are not true polyps but simply repl'''."", adjacent ulceration, undermining the mucosa will, ; II " " lormation . Even when the disease is in remissioll, II lilt" persist as elevated areas between the healeej alli ll ,I,i< I nreviously ulcerated mucosa .
11.1 I III OlvortlculaJ disease. r hi:~ :l lHl rl iMI I " II II, I" l " 1""'"11 111 '. IIIIW IWll il irnO!l1 pHItI IIOI IIIW:. "I " 'Vlll i h Iltlll
ry, ,II ,,, W. " I, I, d,vf) 1III :lIln l di f)I)(Ull ' 01
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1.1
,II " I '" 11. 11 ~
, "'"'" 111, 1,," 111 1I1111 1y with lI r1vIIIICII'IIII!I" "" "/1111'1 III It I , , I, ,.ilil " ,"11111111',11 I ,II J'i' II II Ii , :1111. 1')( I'" ,,,1 \I I Ill" "," I' I, " '"" I , " " lli lill
",' 'j
II
II
I !" i l
HI IIII II I H I
3 Alimentary System
lin nl,pondlcltls. This appendix has been sectionea
til 11(1\1:; intraluminal and intramural purulent II.. , I w, ll, (~"ngestion and haemorrhage in the wall and
I I IIIW, I j
r II i ~ I H
I. II
Fig. 3.36 Ulcerative colitis. The distal portion of this rectum has a granular, almost velvety, appearance with haemorrhage and innumerable shallow ulcers. The proximal margin (above) appears normal. Ulcerative colitis is an idio pathic disease, pre dominantly of young adults, which always involves the rectum and aHects the proximal large bowel in continuity. It is a ch ronic relapsing condition primarily affecting the mucosa . It is assoc iated with HLA B-27 and may be com plicated by toxic megacolon, per foration and the development of adenocarcinoma.
Fig.3.37 Pseudo-polyps in ulcerative colitis. Th is segment of large bowel shows intense mucosal congestion and , in addition, the mucosa is 'thrown up' into innumerable irregular polypoid pro trusions. These are not true polyps but simply represent the effects of adjacent ulceration, undermining the mucosa with granulation tissue formation . Even when the disease is in remission , these tags may persist as elevated areas between the healed atrophic foci of previously ulcerated mucosa .
Fig.3.39 Diverticulitis. This sigmoid colon has been opened to show mucosal congestion associated with a florid, serosal, fibrino purulent exudate . In th e lumen, the ostia of severa l diverticula are vi sible . Inflammation complicating dive rt icular disease results from mucosal ulceration due Ie inspissation of faecal material within diver ticula . Diverticulitis can be either acute or chronic and may give rise to fibrosis or perforation .
Ig. 3.38 Diverticular ijlsease. This sigmoid colon has been "PllnClcl to show two almost parallel rows of diverticular ostia. In the w, ,',lorn World, diverticular disease of the colon is extremely , ""IIItJn, particularly with advancing age. It results from the eHects of III' '" .II.od intraluminal pressure consequent upon the peristaltic con 1' <11 110 111 ' : roquired to propel the more viscid or solid faecal material , , hi li
Fig. 3.40 Chronic ischaemic colitis. This large bowel has been opened from behind to show an area of mucosal congestion assoc iated with fibrous thi ckening of the bowel wall and stricture formation. The appearance is typical of long-standing relative isc haemia which usually results from mesenteric arterial disease without complete occlusion (cf. Fig .3.30) . Commonly, ischaemic colitis is complicated by bacterial infection and progressive fibrosis: as a consequence , the macroscopical appearances can be confused with those of inflammatory bowel disease or malignancy.
Ii
II" ,,,, .0 1\ II IU m a .
32
3 Alimentary System Fig. 3.41 Pseudo membranous colitis. This close· up view of large bowel mucosa shows numerous small , raised , yellowish plaques The appearance is virtually diag· nostic of pseudomembranous colitis and is usually found in the left side of the colon . The con· dition occurs most often follow· ing a course of antibiotics, which, in altering the natural flora and suscep tibility of the colon to bacterial colonisation , allows infection wi tt> erostridium difficile (a Gram-positive anaerobe) and the elaboration of its potent exotoxin to occur.
CLASSIFICATION OF-LARGE
Fig.:!.
B~WEL PO~~
adcn lll ~
eX; If"I'''] <:l< jt ' l u fll
I
Anomalous mucosal fold
fru, II II" tile IlII'f'
'Metaplastic' (hyperplastic)
(UPI' ''' ) sp,', ,",,) hl1!, II.., 1 to:,IH 'w !
Inflammatory pseudo-polyp Lymphoid
101 ,1 ,101 1. '1 ap i II '.1 101 SIlJ.,III II pl:" ,I " I lalll,'1 1i1
juvenile Hamartomatous
Peutz-Jeghers
(;(lll ' " " "
tubular Neoplastic (adenoma)
~()(: I! i I V
wlH,llll
tubulo-villous
II
'lill', IIII' V;III,II II '1 pl"',III I '
villous
"''''' Illy I.
Fig. 3.43 Classification of large bowel pOlyps.
3<; 11111 1 1
11 ,I.Ov il "
Fig.3.42 Amoebic dysentery. The colon shows irregular foci of mucosal congestion and swelling with adjacent areas of ulceration. Amoebic dysentery results from infection with the protozoon Entamoeba histolytica, which is endemic in the tropics After inges tion, the organisms invade the bowel wall and cause submucosill necrosis, which results in 'flask-shaped' ulcers with sparing of the overlying mucosa in the early stages . Compl ications include chronic infection, with fibrosis or exuberant formation of granulation tissue (the 'amoeboma'), and infection of the portal venous system (see FigAA).
33
Fig. 3.44 'Meta plastic' polyp. Th is close-up view of large bowel mucosa shows a very small, pale, polyp oid nodule situated on the crest of one of the mucosal folds . 'Metaplastic' polyps are, in fact, hyper plasti c lesions show ing an increased cell lurnover. They occur in the large bowel and may be found at any age, but are espe cially common from the 5th decade onward s. Th ey are usually mul tiple, small, flat or sessi le and arise most often in the rectu m. They have no malignant potential.
f1,"I1'I ,II
P'I'
Il h" l!
Flg.3.46 Villous adenoma. This example btl'l 1)11"" I ,Ii, race to show that it is a large, broad-bassrl :1,'" ', 01,, 1"', 1 whi c h numerous irregular papillary frond s projoCi N, II .. " Iflarq ins of this polyp are ill-defined , This vari~1f '1< ,1' 1111 'l 'iI '"<)st com mon in the rectum , It tend s to be larfl'" ,lI il l' ,111 "' Jv()re dysplasia than the tubular adenoma 111\(1, ,", ','" ,I , , 11I ' 1I1'1()nly progresses to adenocarcinoma V,II,.",'"" h"" ",' :tllm may somelimes secrele large amOllnl ' , 01I" ,I ", ,I ,01 1"" n,n, giving rise to symptoms of hypokflli 1( 1" 11,' , II I'VI ' 1IIIII'"';tcm,a . ' III
3 Alimentary System Fig.3.45 Tubular adenoma. A typical example of a tubular adenoma projecting from the mucosa of the large bowel (upper) . A separate specimen (lower) has been bisected to show its smooth, lobulated appearance and sm all pedi cle Neo plastic polyps of the large bowel are very common in We stern society, particularly' with increasing age. This most frequent variant of a neo plastic polyp is usually less than 3cm in diameter. They are often multiple and are all pre-malignant.
.:,lf ICATION OF LARGE BOWEL POLYPS I,d, 1I1 ~1 mucosal fold
pI. I; ,IIC' (hyperplastic) II II.(lur y pseudo-polyp
1111111 1
juvenile Ill lITlrltous
Peut:;:-Jeghers tubular
I,,·,II< : 1111 1l d)
tubulo-villous villous
Ilflcation of large bowel pOlypS. Fig. 3.44 'Meta plastic' polyp. This close-up view of large bowel mucosa shows a very small. pale. polypoid nodule situated on the crest of one of the mucosal folds . 'Metaplastic' polyps are, in fact. hyper· plastic lesions show ing an increased cell turnover They oc cur in the large bowel and may be found at any age. but are especially common from the 5th decade onwards. They are usually multiple, small . flat or sessile and arise most often in the rectum. They have no malignant
potential.
'H . 3.46 Villous adenoma. This example has been photographed "/ 11, ,,; 0 to show th at it is a large . broad-based sessile lesion from N illi II III ,rne rous irregular papillary fronds project. Note that the I""/ filII " of this polyp are ill-defined. This variant of neoplastiC polyp is ,, " 1'.1 , III limon in the rectum. It tends to be larger and show more , lV l ' ''' dv~>p lasia than the tubular adenoma and, as such , more , " 11111" II ilv f)rogresses to adenocarcinoma. Villous adenomas of the 1" 1 IIIIIIIII ; IV sometimes secrete large amounts of potassium or III il II I 111 1. I living rise to symptoms of hypokalaemia or hypoa l 1,1111 ii I H II ',lll1i,1 ,
Fig.3.47 Familial pofyposis coli . This segm ent of large bowel is covered in numerous tubular adenomas of va rying size . Pol yposis coli is a rare autosomal dominant inherited condition in which patients develop hundreds of large bowel adenomas, usually in the 2nd and 3rd decades. Close sc reening of all famil y members is Obligatory since, if these patients are left untreated, all will develop one or more adenocarcinomas over a period of 10-20 years . Despite such efforts. up to 40% of cases have a colonic carcinoma at presentation.
Fig.3.48 Ulcerating rectal carcinoma. The distal end of this opened rectum (left) shows an ulcerated tumour with irregular rolled edges. A similar tumour (right) has been sectioned to show penetra tion of the muscle coat and a lymph node containing metastatic tumour is visible in the mesenteric fat. Adenocarcinoma of the large bowel is the second commonest ca use 01 death from malignancy in Britain, even though up to 45% of patient s are cured . It arises most often in the left side of the bowel. There is a familial tenden cy and postulated aetiological factors inctude a low fibre diet, a high fat diet and a dietary alteration in bile salt metabolism. Prognosis is directly related to staging (see Fig . 3.52)
34
3 Alimentary System Fig. 3.49 Fungating rectal carcinoma. This is an abdomino perineal resection specimen showing a 'cauliflower' fungat ing tumour, in the distal rectum, which has been bisected to emphasise its polypoid mode of growth . Th is macro scopical variant of large bowel adeno carcinoma is com paratively uncommon and is usually histologically well diHerentiated .
Fig. 3.51 Caecal carcinoma. The terminal ileum and caecum have been dissected to show three separate lesions arising in the proximal caecal mucosa. The largest (left) is an ulcerated adenocarcinoma while the other two are neoplastic polyps . Adenocarcinoma of the caecum is commoner in females and , owing to the distensibility 01 the caecum , g ives rise to symptoms less often. Insidious blood loss, possibly with melaena, may lead to presentation as iron-deficiency anaemia. Th is specimen demonstrates the frequency with wh ich neoplastic polyps and carcinoma are found in the same specimen .
Fig.4.1 Polycystic liver. The anterior surface 011111 , II v. numerous, multiloculated subcapsular cysts, prodOI l1 I11, lobe , On the right, a separate case shows the c ui ~ .1111. Ii I ance, Polycystic disease of the liver is an inherit o( I ,II '" " dominant condition, frequently associated with :1( lIliI",,' disease. In general it does not impair live r funcllOl1 1\ ' ,II usually less marked, appearance may be seen in i ;Olll I' fibrosis .
DUKES' STAGING OF COLORECTAL ADENOCARCINOMA
Stage
A
B
C Lymph node metastases
Extent of tumour
Confined to bowel wall
Invasion through bowel wall
5 - year survival
90%
65%
20%
mucosa muscularis mucosae
Fig.3.50 Annular stenosing rectal carcinoma. This is another abd omino-perineal resection specimen. The tumour can be seen to encircle the entire lumen of the bowel and shows central ulceration . Circumferential spread may be faci litated by extension of the tumour through submucosal lymphatics. This macroscopical variant is par ticularly likely to give rise to large bowel.obstruction with prox imal dilatation, ste rcoral ulceration and possibl e perforation.
35
muscularis propria serosa fat lymph nodes
Fig.3.52 Dukes' staging , conceived by Cuthberl Dukes, St. Mark'!"; Hospital , London .
IIU, 4,2 Massive hepatic necrosis. Til e liVl)1 I:. I ""I_11111 1 111 "111,1111 , Ihe capsule has a wrinklcri, I a lil t'l l 1. )( 1'.11 111'1 " till ~ I, j'.I"\I' · tr" polic necros is is unCO lTl1l10ll PUI r: : " '11 ' ,1 I,. " III,; I. .1II ' " 1111 1 wllh lulmillanl viraIIIOploXII ,."" " I! I!, 111111111' , d:,Ir" lolllflll(!, "IOlllyld"pl ldlld '''UIlI.1 /1I 1 III' \ ! iii ." II ' 11I y J II It II [ II It! ; In II,; Il clllll lf(~ lillli II I 1 Ulpll lIy ',11111 11 \111111
4 Hepatobiliary System
carcinoma. The terminal ileum and caecum have show three separate lesions arising in the proximal largesl (left) is an ulcerated adenocarcinoma neoplastic polyps. Adenocarcinoma of the er in females and, owing to the distensibility of the to symploms less often. Insidious blood loss, may lead to presentation as iron-deficiency \ecimen demonstrates the frequency with which Ii and carcinoma are found in the same specimen .
Fig.4.1 Polycystic liver. The anterior surface of Ihe liver (left) shows numerous, multiloculated subcapsular cysts , predominanlly in the left lobe. On the right, a separate case shows the cut-surface appear ance. Polycystic disease of the liver is an inherited autosomal dominanl condition , frequently associated with adult polycystic renal disease . In general it does not impair liver function. A similar, though usually less marked, appearance may be seen in congenital hepatic fibrosis .
Fig.4.3 Hepatic abscess. Within the liver parenchyma is a large abscess cavity, lined by purulent material, and showing central necrosis. There is also an adjacent smaller lesion. Hepatic abscesses most often complicate suppurative cholangitis or portal pyaemia, as may be seen in diverticulitis or appendicitis. Such abscesses are commonly multiple and are usually due to infection by gut flora such as Gram-negative or anaerobic bacteria.
iU,4.2 Massive hepatic necrosis. The liver is pink and mildly I q ' " 11\; \1 1: the capsule has a wrinkled, rather loose appearance. , .. IV" Ilfl patic necrosis is uncommon but is most frequently 11 '" loIllld wllh fulminant viral hepatitis (usually hepatitis B or non-A , '" ,I I 11\. 1IIIIOy also be caused by other hepatotoxic agents including I" " ,1 1111 11, n.s halothane, methyldopa and isoniazid. The prognosis is I' I ,II V IIUOI and acute hepatic failure rapidly supervenes.
Fig.4.4 Hepatic amoebic 'abscess'. In the posterior aspect of the right lobe is a large necrotic cavity showing surrounding fibrosis. The contents of the cavity are said to bear some resemblance to anchovy sauce. Hepatic involvement by Entamoeba histolytica occurs via the portal venous system (see Fig .3.42) and may be seen in up to 30% of cases of amoebiasis. Necrosis, as seen here, is caused by the protozoa and is the commonest manifestation but it should be noted that no true suppuration occurs.
GING OF COLORECTAL ADENOCARCINOMA
A
B
C
Confinecito bowel wall
Invasion through bowel wall
Lymph node metastases
90°/,
65%
20%
~ ' staging, conceived by Cuthbert Dukes, St. Mark's I,
i"
36
4 Hepatobiliary System
Fig.4.5 Hepatic hydatid cyst. A transverse section through this liver shows a well circumscribed loculated fibrous cyst. Hydatid d isease is due to infestation by the tapeworm Echinococcus granulosus. and is seen most often in sheep-farming communities . Spread to the liver occurs via the portal system from the duodenum and affecls at least 50% of cases. Such cysts are usually solitary, are found most often in the right lobe and contain many daughter cysts with brood capsules and scolices. The case here appears to be 'burnt-out'.
Fig.4.7 Hepatic Zahn infarct. In this close-up view, an approx imately wedge-shaped area of subcapsular parenchyma shows a congested pseudo-infarct with slight concavity of the overlying capsule. This is the typicat appearance which results from throm bosis of a portal vein radicle, usually as a consequence of a small embolus or compression by tumour. The hepatic arterial supply prevents true infarction in such cases, but parenchymal atrophy and sinusoidal congestion occur .
Fig.4.6 Passive hepatic venous congestion. The cut surface of the liver has a variegated appearance, reminiscent of a nutmeg , with small multi focal areas of congestion surrounded by a rim of pale tissue. This appearance is the result of chronic congestive cardiac failure with centrilobular venous congestion and atrophy, or occasionally fatty change, of the adjacent parenchyma. In some cases there may be associated fibrosis, but the development of true cirrhosis is an extremely rare complication .
Fig.4.8 Portal vein thrombosis. A large branch of the portal vein is totally occluded by thrombus. Note that the liver parenchyma shows florid macronodular cirrhosis. Portal vein thrombosis is most often associated with either local venous obstruction by a neoplasm, intra-abdominal sepsis or recent abdominal surgery , While passive splenic congestion commonly ensues, true hepatic infarction does not occur unless the blood supply from the hepatic artery is also compromised.
37
Fig.4.9 Budd-Chiari syndrome. The liver pur I '11, I,v' " an exaggerated 'nutmeg' pattern (see Fig A ,(;) .1 1111 >,'IV of the hepatic vein are occluded by thromULJ!: II IlId'I'I I (bottom) occupies the lumen of the inferim VO l I;, , ~ ; 'V" primary leiomyosarcoma . This rare syndrorrll ! I!, tlr'" II, the prinCipal hepatic veins or inferior vena L. , IV i I, , I'" I II i endophlebitis, obstruction by tumour eith(;l f II "", II V (II secondary or adjacent, or associated willI I II ,lye.yIl I' '' '11
4 Hepatobiliary System
[
CAUSES OF HEPATIC FATTY CHANGE
Alcohol abuse Starvation/malnutrition
Diabetes mellitus
Glycogen storage diseases
Galactosaemia infarct . In this clo se·up v iew. an approx· area of subca psu lar parenchyma shows a with slight concavi ty of the overlyi ng appearance which results from throm radicle. usually as a consequence of a sm all by tumour The hepatic arterial supply in such cases. but parenchymal atrophy and
acute idiopathic fatty change Pregnancy fatty change with hyperemesis
Severe systemic infection
Pathological obesity
1
Cystic fibrosis
,
,
thrombosis. A large branch of the portal vei n is thrombus. Note that the li ver parenchyma shows Cirrhosis. Portal vein thrombosis is most often local venous obstruction by a neoplasm, or recent abdomin al surgery. While passive romrnon ly ensues, true hepatic infarction does blood supply from the hepatic artery is also
Drugs, especially tetracycline
Flg.4.9 Budd-Chiarl syndrome. The liver parenchyma (top) shows 0111 <)xaggera ted 'nutmeg' pattern (see Fig.4.6) and several tributaries I1ltllu hepatic vein are occlu ded by throm bus. A mass of tumour (11I)1I 0m) occupies the lumen of the in ferio r vena cava. This was a I "III li lly leiom yosarcoma. Thi s rare syndrome is due to throm bosis of II,,· I'l'Inc ipal hepat ic ve ins or inferior ve na cava, and may be due to ' " " lill liliobilis, obstruction by tumour either primary (as in thi s case ), . ,' II , l/ Iti llry or adjacent , or associated with polycythaemia rubra ve ra .
Chemical toxins, such as carbon tetrachloride
Reye's syndrome Fig.4.10 Causes of hepatic fatty change ,
38
.....
4 Hepatobiliary System
CAUSES OF CIRRHOSIS -
Alcohol abuse Post-viral (hepatitis B/non-A, non-B/o agent)
f
Biliary (primary or secondary) Haemochromatosis
Fig.4.11 Hepatic fatty change. On the left, the liver parenchyma is diHusely yellowish and, on the right, a portion of a similar liver has been stained with Scharlach R which stains the fat orange-red . Fatty change represents excessive cytoplasmic accumulation of neutral lipid and triglyceride and, as a consequence of its important meta bolic role, the liver is particularly liable to be aHected. Alcohol abuse is the commonest cause in the Western World but, on a global scale, malnutrition is probably the single most important factor.
Wilson's disease Q1 -
antitrypsin deficiency
Indian childhood Metabolic storage disorders Drugs (methyldopa, isoniazid.) -
-
-
-
--
_
._ -
Fig.4.13 Causes of cirrhosis. 10-15% of cases remain idiopathic .
.-
.
Fig.4.15 Macronodular cirrhosis. This close-lip Vi" surface of the liver to be composed of large (>:lrlllll). I varying size, each separated by dense fibrous 1,111" II' macronodular pattern is a feature of post-viral (11' :lIlI lI v cirrhosis and Wilson's disease. Note, however, 111111' " ' livers show a mixed pattern, irrespective of aetiolnl IV
: ....
_':' ?,:;(;,~,~ib{~~::~' f:;· ,,~... &,,, . . :~.., ,, ..
.,,<~:~~:,:;"'J" __ ",..~......!__.~~1<·/~'<'.l-,:,t~. ; /,!!~" "'!' >~ .. " /~~~ .. ~ """!l\•. 4''''·';·'-··;':'' A".·¥- . ';' .
,",.
.~..;:~~~l~'~~\~.~ . . 'lt,;';~;i~l l¥,'"~.:~'."'(:-''.~.;;.:.;,;,~<"e;~:~L;, .I'••
/'< '",,'h.';': .
.... ,~: ),_.~,: . d'c·... '.11; •.. ~';;'. .'..... . ,r.~." ' .-' ~""6, '-, ~ ..~!I;..... ~"'. , " ....'-"'"1!::':''''' ~...,.. "",. &i"A.;;...,•. . ' ~,_:""~'" ',""", ',"-.~ 'A,' . ~ """_.~iF.~.'~ ':<'i:1i.'':'P';;~ ..'>''.'·:'·~''':::-··'/·~~.1·-:>'\ :i:';':~:;~''''::' 'r',
.,.
'<' , •..".'.,. ,'_",'"
<~ .'.:Ii.'" ,., .,a ' ,,",' ~'. .•..' ".v,"- ,'k"""'" "~
." . ..' .;,J:",,"'"'."''''',
Fig.4.12 Hepatic amyloid deposition. The liver parenchyma, originally rather waxy in appearance, has been stained with Congo Red to show extensive deposition of amyloid, particularly in the mid-zone of the lobules. Hepatic amyloidosis is most often secondary in type, being composed of serum amyloid A protein. Common' causes of secondary amyloidosis include chronic infection or chronic inflammatory disorders such as rheumatoid arthritis. While the liver may become enlarged and firm , functional impairment is rare despite a degree of parenchymal atrophy.
39
;...
~"'~li;::'~:'.!~"
,
Fig.4.14 Micronodular cirrhosis. The liver is small and the cut surface shows multiple small, pale nodules which are rather uniform in appearance. Micronodular cirrhosis (nodules < 3mm in diameter) is classically seen in alcohol abuse and haemochromatosis. World wide, alcohol is by far the most common cause of cirrhosis and the incidence is increasing . Complications include portal hypertension, splenomegaly, ascites, encephalopathy and clotting abnormalities.
Flg.4.16 Biliary cirrhosis. The cut surface flf 1I1I'.ItV' i tnicronodular pattern, associated with markor l \1" "II I I • Ililiary cirrhosis, which is uncommon, is most ull< IIII" III I his is an idiopathic condition which usually cilluch II ill Inmales, is associated with anti-mitrochond l inl rllllil" I' J I ,c; autoimmune in origin. Very rarely, a simil w . 'PI " 1/ 11 ' I !loc n secondary to long-standing, extra-hAp"t,, ' "I '1.llt l ,tlllCoviscidosis.
4 Hepatobiliary System
OF CIRRHOSIS
(hepatitis SInon-A. non-B /o agent)
Fig.4.15 Macronodular cirrhosis. This close-up view shows the cut surface of the liver to be composed of large (>3mm), pale nodules of varying size, each separated by dense fibrous bands. Typically, a macronodular pattern is a feature of post-viral (usually Hepatitis B) cirrhosis and Wilson's disease. Note, however, that many cirrhotic livers show a mixed pattern, irrespective of aetiology.
cirrhosis. The liver is small and the cut "I~ small. pale nodules which are rather uniform
lar cirrhosis (nodules < 3mm in diameter) I abuse and haemochromatosis . World most common cause of cirrhosis and the Compflcations include portal hypertension, encephalopathy and clotting abnormalities.
Flg.4.16 Biliary cirrhosis. The cut surface of this liver shows a IIliCronodular pattern, associated with marked green bile-staining. I Iiliary cirrhosis, which is uncommon, is most often primary in type. 1111:; is an idiopathic condition which usually aHects middle-aged 1III110les, is associated with anti-mitrochondrial antibodies and may I II) IllJtoimm une in origin. Very rarely, a similar appearance may be 'It .un secondary to long-standing, extra-hepatic obstruction or "IIII .oviscidosis.
Fig.4.17 Haemochromatosls. The pancreas (right) and, to a lesser extent, the cut surface of the liver show deep brown pig mentation, due largely to excessive deposition of haemosiderin. Idiopathic haemochromatosis is an auto somal dominantly-inherited condition, characterised by defective iron metabolism, which results in massive iron de position, particularly in the live r, pancreas , heart , adrenals and skin . Males are most often affected and typica lly present in middle age. Complications include the development of cirrhosis, hepatocellular carcinoma, diabetes mellitus, cardiac failure and Addison's disease.
Fig.4.18 Hepatoma with micronodular cirrhosis. The live r is diffusely nodular but, in addition, two irregular areas of pale , neo plastic tissue are apparent. The larger, central portion of tumour has invaded the main hepatic vei n. Hepatoma, (primary hepatocellular carcinoma) is not uncommon and is seen most often in Africa and the Far East. A high proportion of'cases are associated with pre-existent cirrhosis, particularly alcoholic, viral and that associated with haemochromatosis. Other known causes include aflatoxins (from mould y grain) and various alkaloids e.g . from herbal teas.
40
.........
4 Hepatobiliary System
Fig.4.19 Multinodular hepatoma. The liver parenchyma is diffusely replaced by numerous greenish nodules of tumour. Between these nodules , the uninvolved hepatic tissue shows a micronodular cirrhosis . It is unknown whether this pattern of hepatocellular carcinoma represents multifocality of origin or intrahepatic meta stasis. Occasionally hepatomas may display a diffuse micronodular infiltrative appearance wh'lch may be difficult to distinguish macro scopically from cirrhosis . The aetiological factors and proqnosis of hepatoma are identical , irrespective of the gross appearance.
Fig_4_20 Angiosarcoma of liver. The hepatic parenchyma is largely replaced by a diffuse haemorrhagic neoplasm in which multiple small vascular channels are visible. Hepatic angiosarcoma is rare and may arise either in infants or, more usually, in adults. Adult cases may be associated with previous exposure to the contrast medium , Thoro trast, or to vinyl chloride monomer in the plastics industry. In general the prognosis is extremely poor.
41
-
Fig_4.21 Hepatic metastases. Multiple irregular nodules of pale secondary tumour are randomly distributed in the parenchyma of this otherwise normal liver. Metastatic involvement of the liver is extremely common and occurs most often in association with primary tumours drained by the portat venous system , particularly gastro-intestinal adenocarcinomas. The liver is also a very common site of secondary spread from carcinomas of the bronchus and breast and malignant melanoma. Metastases are very uncommon in cirrhotic tivers, probably as a consequence of alterations in hepatic blood flow.
Fig.4.22 Hepatic involvement by lymphoma. Throughout the liver parenchyma there is diffuse infiltration by innumerable, small,pale deposits of lymphomatous tissue . This is the typical appearance of disseminated lymphoma, the liver being involved in up to 50% of cases of either the Hodgkin's or non-Hodgkin's type . Very rarely a lymphoma may arise primarily in the liver .
Fig_4.23 Acute cholecystitis. This gallbladder 11. 1'.1 11 show intense congestion and ulceration of the $IIJI.I< " ' The serosal surface is also congested and the rUII1IIII1 " purulent exudate are visible. Acute cholecystiti s I: . 1.111". association with gallstones (see Fig.4 .2S), partic ul.lliv II duct is obstructed. Occasional cases may occur I"IYI" septicaemia Possible complications include pr:rle", ,Ie, II itis.
Flg.4.24 Chronic cholecystitis. The gallbl;J1 Ie I, 'I II, V' i' I)nel its wall is thickened and fibrotic . The mur,nt.cl i '.II,l.e. co ngested and there are several small,ntrahrllllr ll ,l 1/,,11 , I'I.ppearances represent the effects of prol oril !elli . eI· " " ,I! ;1tIocks of acute cholecystitis. As such, thp. GI)e'XI'.I, 11 e' •I, Il' extremely common .
4 Hepatobiliary System
metastases. Multiple irregular nodules of pale are randomly distributed in the parenchyma of this liver. Metastatic involvement of the liver is extremely Irs most often in association with primary tumours rtal venous system , particularly gastrO-intestinal , The liver is also a very common site of secondary ",m."S of the bronchus and breast and malignant are very uncommon in cirrhotic livers, ence of alterations in hepatic blood flow .
involvement by lymphoma. Throughout the liver 'e; diffuse infiltration by innumerable, small,pale I(Jl n910us tissue. This is the typical appearance of IIp''()ma, the liver being involved in up to 50% of t I, lctg kin's or non-Hodgkin 's type. Very rarely a ml!' primarily in the liver . !
Fig-4-23 Acute cholecystitis. This gallbladder has been opened to show intense congestion and ulceration of the surface epithelium The serosal surface is also congested and the remnants of a fibrino purulent exudate are visible. Acute cholecystitis is seen most often in association with gallstones (see Fig.4 .25), particularly if the cystic duct is obstructed . Occasional cases may occur in typhoid fever or septicaemia . Possible complications include perforation and periton itis.
Fig.4.24 Chronic cholecystitis. The gallbladder is very shrunken [lnd its wall is thickened and fibrotic. The mucosal surface is ~ongested and there are several small intraluminal gallstones. These appearances represent the effects of prolonged , usually intermittent , :'Ittacks of acute cholecystitis As such, the coexistence of gallstones it extremely common.
Fig.4.25 Cholelithiasis. This opened gallbladder contains two large mixed gallstones . Note the marked thickening of the gallbladder wall and the stigmata of acute inflammation of the mucosa. Gallstones are extremel y common and may be of cholesterol, bile pigment or mixed type Adults , particularly women, are most often affected . Alterations in the bile content are probably the most important aetiological factors . Complications include cholecystitis, biliary obstruction, cholangitis, and stricture of the common bile duct.
Fig.4.26 Gallstones in the common bile duct. Multiple mixed gallstones are visible within both the gallbladder (below) and the grossly dilated cystic and common bile ducts . The passage of such stones into the bile ducts may result in biliary colic or obstructive jaundice, sometimes complicated by ascending infection. Damage to the duct wall can lead to stricture formation or, occasionally, utceration into the duodenum which may later be succeeded by gallstone ileus.
42
4 Hepatobiliary System Fig.4.27 Cholesterolosis. The mucosa of the gallbladder is rather congested and is studded with multiple small, yellow flecks; th is appearance is known as the'straw berry' gallbladder. Cholesterolosis is a very common con dition characterised by deposition of lipid beneath the mucosa. It rarely causes symptoms apd is thought to be due to a localised abnormality of cholesterol metabolism or absorption.
Fig.4.28 Carcinoma of gallbladder. This dilated gallbladder has been opened to show a fungating, partly papillary tumour arising in the fundus. In the body are three small pigment stones'. Adeno carcinoma of the gallbladder is uncommon and occurs most often in old age, predominantly in females . In the majority of cases there is a long-standing history of preceding cholelithiasis and cholecystitis . Invasion of the adjacent liver is an early feature, often resulting in inoperability and a poor prognosis.
43
Fig.4.29 Cholangiocarcinoma. Arising at the confluence of the right and left main hepatic ducts is a pale, locally infiltrative tumour. Each of the ducts is markedly dilated , Cholangio carcinoma (primary bile duct carcinoma) is more commonlY extrahepatic than intrahepatic in origin This tumour typically arises in late adult life , in either sex, and shows no association with cholelithiasis (ct . carcinoma of the gallbladder). In some Orientals, biliary infestation with the fluke Clonorchis sinensis is a predisposing factor. Fig.4.31 Chronic pancreatitis. The pancreatic li:;,;rll' ',1" irregular scarring; the pancreatic duct (right) is dil;rl. ,II -III. pale calculus Chronic pancreatitis may take two" lilli' , 11" type due to repeated acute attacks and the de nUV( I I IlIl lIl Both are sign ificantly related to alcohol abuse althulIlI" 110 coexistent duct obstruction or dysfunction . It is unclr ll il wi formation predisposes to , or is a consequence of. (;llIlIl1lo pancreatitis , Flg.4.30 Acute pancreatitis. Between the porta hepatis (above) and an adjacent loop of small bowel there is extensive necrosis of mesenteric fat , the greyish areas re presenting necrotic pancreati c tissue. Acute pancreatitis is relatively common and is most often associated with alcohol abuse or biliary tract disease, such as choleli thiasis. Middle-aged adults are typically affected and the pathological features are attribut able to extensive destruction by released pancreatic enzymes.
Fig.4.32 Congenital pancreatic cyst. Projectinq fl. "" III surface of this otherwise normal pancreas is a small, 'H I li II loculated cyst. Pancreatic cysts may be congenit al (. 111'11 1 with renal polycystic disease or cerebral angioma!":), , II '1" pancreatic duct obstruction (retention cyst), neopla;,tll (I, a cystadenoma or cystadenocarcinoma) or false ill "-11111' . necrotic areas complicating acute pancreatitis · IJ';( Hldl II
4 Hepatobiliary System
Fig.4.31 Chronic pancreatitis. The pancreatic tissue shows marked irregular scarring; the pancreatic duct (right) is dilated and contains a pale calculus. Chronic pancreatitis may take two forms: the relapsing type due to repeated acute attacks and the de novo chronic type . Both are significantly related to alcohol abuse although there may be coexistent duct obstruction or dysfunction. It is unclear whether stone formation predisposes to, or is a consequence of . chronic pancreatitis
Fig.4.32 Congenital pancreatic cyst. Projecting from the superior surface of this otherwise normal pancreas is a small, smooth multi· loculated cyst. Pancreatic cysts may be congenital (often assocjated with renal polycystic disease or cerebral angiomas), acquired due to pancreatic duct obstruction (retention cyst), neoplastic (being either a cystadenoma or cystadenocarcinoma) or false in nature (walled·off necrotic areas complicating acute pancreatitis - pseudocyst).
Fig.4.33 Carcinoma of the head of the pancreas. In the concavity of this loop of duodenum, the head of the pancreas is enlarged and totally replaced by a pale mass of tumour , just above which the grossly dilated (opened) common bile duct is evident. Up to 70% of exocrine pancreatic adenocarcinomas arise in the head of the gland; males are most often affected , usually in the 6th and 7th decades. Hepatobiliary failure with j"lUndice, due to bile duct ob· struction , inoperability and the complications of radical surgery result in a very poor prognosis, although metastases may be absent or minimal.
Fig.4.34 Malignant islet cell tumour. This pancreas contains multiple, irregular pale masses of tumour, the largest of which is in the tail of the gland. The majority of islet cell tumours are, in fact , benign, the commonest being an adenoma of rl cells giving rise to hyper insulinism. Other adenomas may secrete glucagon (0. cells), somatostatin (8 cells), gastrin (perhaps from 8 cells), pancreatic polypeptide or vasoactive intestinal polypeptide . Islet cell tumours may form part of the Type I Multiple Endocrine Neoplasia Syndrome. Up to 60% of gastrin-secreting.tumours are malignant. In this case the re has been extensive intrapancreatic spread .
44
5 Breast Fig.5.1
Mammillary fistula.
This section through areolar skin and adjacent breast tissue shows an in flamed fistulous tract, communicat ing with a small abscess cavity (left). Such a fistula most often occurs as a complication of re current pyogenic mastitis and may be associated with duct obstruction or duct ectasia. Lactation is a common predis posing factor .
Fig.5.2 Fibrocystic disease. This breast tissue has been sectioned to show diffuse fibrosis and multiple cysts of variable size . Thi s condition is extremely common, is often bilateral and usually affects females in the 4th and 5th decades. It is probably due to a disordered or imbalanced re sponse to endogenous sex hormones. Only in those cases showing marked epithelial hyperplasia (epitheliosis) is there thought to be an increased risk of breast carcinoma.
45
Fig.5.3 Fibroadenoma. The specimen consists of a well circumscribed, pale, lobulated tumour which has been 'shelled out' from the adjacent breast tissue at operation. Fibroadenomas are extremely common benign neoplasms, which may be multiple and typically arise between the menarche and the age of 30. They are mobile and rubbery, whence the term 'breast mouse' , and are not related to the development of breast cancer.
Fig.5.4 Fibroadenoma. This is a rather larger example than Fig.5 .3 and demonstrates particularly well the characteristic lobulation that these tumours often show. The lobules are clearly demarcated by pale bands of fibrous tissue. It is worth noting that the historical division of fibroadenomas into intracanalicular and pericanalicular subtypes is probably spurious , since on histological grounds the two patterns almost invariably coexist.
Fig.5.5 Giant fibroadenoma (Phyllodes tumour). Wil l tissue is a large, lobulated mass showing myxoirl. 11111 'I '" cystic loci. These tumours are relatively uncomlllOII, III r nantly in the 5th and 6th decades and may attaill il l l lO lli l are probably unrelated to the more common fibrnf "I, ,'I, I page 45), Despite their obsolete name, cystOS8 n, Olllu I'" about 5% behave in a malignant fashion,
Fig.5.6 Duct papillomata. A transverse secti()11 /1 11111111 ' tissue shows a collection of cystic ally dilated dIICI· ., il l " I the lumen is obstructed by soft, brownish, papill:IlY Ii '.' ilil represent multiple duct papillomata which typi ca lly, 11 1'11 lactiferous duct. near the nipple, Duct papiliomAI!1.11" , I" usually solitary. arise in middle-aged women 81 11 I . 11 1' 1" " benign lesions. They frequently give rise to a blo( HI ',/., 11 1 charge. causing clinical confusion with carcinonili
5
Bre~t
5 Brea.st
Fig.5.5 Giant fibroadenoma (Phyllodes tumour). Within the breast tissue is a large, lobulated mass showing myxoid, haemorrhagic and cystic foci. These tumours are relatively uncommon, occur predomi nantly in the 5th and 6th decades and may attain a great size . They are probably unrelated to the more common fibroadenomas (see page 45). Despite their obsolete name, cystosarcoma phyllodes, only about 5% behave in a malignant fashion .
Fig.5.7 Intraduct carcinoma. On the cut surface of this breast tissue multiple small ducts, obstructed by pale tumour, are visible. The tumour appears to ooze out, rather like toothpaste - an appearance also known as comedo carcinoma . lntraduct carcinoma represents the pre-invasive stage of breast cancer of duct origin: there is also an intralobular equivalent which may be bilateral in up to 40% of patients. Excision at this stage, in the absence of histological invasion, is curative.
TOPOGRAPHICAL DISTRIBUTION
OF FEMALE BREAST CANCER
o
axillary tail
upper lateral
quadrant
Fig.5.6 Duct papillomata. A transverse section through this breast tissue shows a collection of cystically dilated ducts, in many of which the lumen is obstructed by soft, brownish, papillary tissue. These represent multiple duct papillomata which typically arise in a major lactiferous duct, near the nipple . Duct papillomata are, however, usually solitary, arise in middle-aged women and are uncommon benign lesions. They frequently give rise to a blood-stained dis charge, causing clinical confusion with carcinoma.
lower lateral
quadrant
areola and nipple
upper medial quadrant
10%
Fig.5.S Topographical distribution of female breast carcinoma.
46
5 Breast Fig.5.9 Scirrhous adeno carcinoma. A cross· section through the breast shows an irregular, pale, stippled mass beneath the retracted nipple. Note the claw·like extensions of tumour and fibrous tissue in the adjacent fat. This is the commonest macroscopical variant of breast cancer, which occurs in about 6% of females , usually in the 5th/6th decades, and is by far the commonest malignancy in women . The aetio· logy is uncertain but lack of previous lactation, geographical factors and epitheliosis seem to be important: 90% are of duct origin and 10% lobular. Lymph node involvement is the most important prognostic factor. 5· year·survival is only 30%, largely due to failure in early diagnosis.
Fig.5.10 Encephaloid adenocarcinoma. Much of this breast is replaced by a large , fairly well circumscribed, lobulated tumour showing focal cystic and haemorrhagic change. The cut surface. resembles cerebral tissue . This macroscopical variant of breast cancer accounts for about 8 % of cases and most often represents the medullary or colloid histological subtypes. Both these types carry a better than average prognosis.
47
Fig.5.11 Ulcerated adenocarcinoma. Above the nipple, the skin of this mastectomy specimen is raised and ulcerated by an underlying carcinoma. Local invasion of the skin by breast cancer is common and may confer a worse prognosis. Cutaneous in vol vement, tocally or at a distant site, may also occur due to lymphatic or haematogenous spread .
Fig.5.12 Carcinoma with nipple retraction. The breast is severely distorted by a large underlying carcinoma , with resulting ulceration and local inflammation. Above the tumour , the nipple is totally retracted within the areola (arrowed). Recent onset of nipple retrac tion may be a very useful clinical sign in the diagnosis of breast cancer, particularly if the underlying tumour is small or impalpable.
Fig.5.13 Paget's disease of the nipple. Th e Ilil '1 111' P I i i i shows an eczematous rash which extends to invl1lvll 1111 1. Mammary Paget's disease represents infiltratioll 0 111 111 '11 , underlying ductal adenocarcinoma, which is prlj!lOIlI III , (even if impalpable) . This is a feature of only abolll I 'L, (II cancers. Skin biopsy is mandatory in all adult CA ~OI , ( ,1 ,11 nipple since mistaken treatment for dermatitis will d iliro v I I surgery and may impair the prognosis.
Fig.5.14 Peau d'orange. The skin of this bred::1 I', III' 'I II I dimpled and pitted , bearing a superficial resemL,llI111 I ' 1' 1 This appearance is seen in advanced breast Calli f" 11111 1 lymphoedema, resulting from lymphatic obstru ~: III )J I I 'v if I tumour cells. Pitting occurs because mammary " hili \'. " ,II innumerable sweat glands.
'5
B n..I ~t
lYMPHATIC SPREAD OF BREAST CANCER
supracl avicular nodes
axillary nodes
mediastinal nodes
~ p l e,
Ihe skin of an underlying 'ff is common menl, locally or '3emal ogenous
cfavicfe
Fig.5,13 Paget's disease of the nipple. The nipple is eroded and shows an eczematous rash which extends to involve the areola. Mammary Pagel's disease represents infiltrat ion of the skin by an underlying ductal adenocarcinoma, which is present in every case (even if impalpable). This is a feature of only about 1 % of breast cancers. Skin biopsy is mandatory in all adult cases of eczema of the nipple since mistaken treatmenl for dermal itis wi ll delay definitive surgery and may impair Ihe p rognosis.
internal thoracic chain :'~st
is severely
; ~~g ulceralion ;~ tolally
~\iPple relrac ~ of breasl ~I mpalpable .
Fig.5,14 Peau d'orange, The skin of this breasl is irregularly dimpled and pitted, bearing a superficial resemblance to orange peel . This appearance is seen in advanced breasl canc er and is due 10 local lymphoedema, resulting from lymphatic obstruction by invasive tumour cells. Pitting occurs because mammary skin is lethered by innumerable sweat glands .
Fig.5,15 lymphatic spread of breast cancer. Lymph node metaslases are present at Ihe time of diagnosis in up to 60% of cases. Local retrograde spread wi thin superficial lymphatics may give rise 10 peau d'orange (see Fig.5 14) or carcinoma-en-cui rasse.
48
6 Lymphoreticular System
5 Breast
Fig.S.16 Non-Hodgkin's lymphoma. The breast is largely replaced by a pale, focally haemorrhagic mass which is well circumscribed and not dissimilar to an encephaloid carcinoma in appearance . Primary Hodgkin's or non-Hodgkin's lymphoma of the breast is un · common but well recognised and is often succeeded by systemic dissemination. The breast may also be secondarily involved by a primary neoplasm arising in lymphoid tissue .
Fig.S.18 Male breast carcinoma. A section through this male breasl shows diffuse replacement by pale tumour with tether ing of the overlying nipple, (centre left) . The pectoral muscle (right) is not involved . Male breast cancer is about 100 times less common than its female counterpart and tends to affect rather older patients . Histologically, the same types are seen in both groups but the prognosis in men is even worse, probably because extensive local invasion occurs at an earlier stage due to the small size of the male breast.
I
CAUSES OF SPLENOMEGALY VASCULAR CONGESTION
Congestive cardi;l(: 1,IIh'i Portal hypertenSioll 11111 I," !'lClflih
Pyogenic INFECTIVE
III . VII 111111 1111.
Non-pyogenic
(lIIal.l'
HAEMATOLOGICAL
Abnormal RBC's (haemolytic anaulIlI,I")
Extramedullary halllll!!1 ., Lipid storage disOi': ,"!' METABOLIC
Glycogen storaga ( 11 ',11, 1 11111 lin .
Primary
lyllll'l , NEOPLASTIC
lo"koll
Secondary
(;, lIdli
AmyloidosiS MISCELLANEOUS
Sarcoidosis
SPLENOMEGALY CLASSIFIED BY WEIGHT MILD < 500g
Acute infection
(li (; ' " 11
Chronic infectioll MODERATE 500-1000g
Haematological
II
0 1 , III '
(;:\11 1,"'.
Amyloidosis Fig.S.17 Gynaecomastia. The se are bilateral subcutaneous mastectomy specimens from a young man: each shows ve ry marked hypertrophy of fibrofatty breast tissue . Gynaecomastia (enlargement . of the male breast) may be physiological, as sometimes occurs at puberty or in old age, or pathological. Causes of the latter include endoc rine disturbances, a va riety of drugs.(e.g. cimetidine). Klinefelter's syndrome and testicular or adrenal tumours . This con dition is unrelated to the development of male breast cancer.
49
MASSIVE >1 000g
Lymphoma, l~lIk : I<'"I1,' or myeloprolih~ lililV Il
"i
Storage disease:, Fig.6.1 Causes of splenomegaly.
6 Lymphoreticular System
I
CAUSES OF SPLENOMEGALY VASCULAR CONGESTION
Congestive cardiac failure Portal hypertension Pyogenic
bacterial septicaemia
Non-pyogenic
TB, viral, fungal, parasitic (malaria)
INFECTIVE
HAEMATOLOG ICAl
Abnormal RBC's (haemolytic anaemias)
Fig.6.2 Passive venous congestion. This enlarged spleen, weighing 740g, is very firm (retaining its shape despite being sliced) and is rather rounded in appearance (being known as a 'cricket ball spleen'). Passive venous congestion is the commonest cause 01 splen omegaly: there is usually only mild enlargement (most often as a consequence of congestive cardiac failure) but greater expansion (as here) may be seen in chronic portal hyper tension (e.g. due to hepatic cirrhosis).
Extramedullary haemopoiesis METABOLIC
NEOPLASTIC
lipid storage diseases Glycogen storage diseases Primary
haemangioma
Secondary
leukaemia
lymphoma
carcinoma MISCEllANEOUS
Amyloidosis Sarcoidosis
~
I SPLENOMEGALY CLASSIFIED BY WEIGHT MilD <500g
I
Acute infection or congestion Chronic infection or congestion
MODERATE 500-1000g
Haematological causes Amyloidosis
MASSIVE >1000g
lymphoma, leukaemia or myeloproliferative disorder Storage diseases
Fig.6.1 Causes of splenomegaly.
Fig.6.4 Chronic perisplenitis. Fig.6.3 Splenic infarction. The splenic capsule is covered Almost the entire spleen shows dull reddish infarction, only those in dense, irregular, hyalinised, areas with a granular cut surface fibrous tissue. This appearance, known as 'sugar-icing' spleen, being spared. Thrombus is may be seen in any case of long clearly visible occluding the splenic artery. Splenic infarction standing splenomegaly or may be embolic in origin, or due associated with chronic pleural to local thrombosis (e.g. compli inflammation. Acute peris plenitiS, characterised by a cating atheroma, sickle cell serosal fibrinous exudate , may anaemia or polyarteritis) or due to torsion. Small infarcts are also occur in cases of septicaemia or overlying a splenic infarct. commonly seen in almost any case of moderate or massive splenomegaly.
50
6 Lymphore ticular System
Fig.6.S Miliary tuberculosis. Scattered throughou t the splenic parenchyma are multiple, tiny, pale nodules resem bling millet seed (hence the name). Miliary tubercu losis represents haematogenous spread of infec tion, most often from a primary focus in the lung (see Chapter 2). Th is vascular dissemination results from encroachment and ulceration of the infective process through a vessel wall: this leads to intrapulmonary spread, in addition to involvement of other organs such as the liver. bone marrow and kidneys.
Fig.6.6 Sarcoidosis. Scattered throughout the splenic parenchyma are coarse pale nod ules, arising mainly in the white pulp . Sarcoidosis is an idiopathic, granulomatous. chronic inflammatory disorder, whic h is commonest in young adults. LymphOid tissue throughout the body is predominantly aHected. al though palpable splenomegaly is seen in only about 20% of cases . Typically. the lungs are also involved and patients often present with respiratory symptoms ; pulmonary inter stitial fibrosis is an occasional complica!ion .
51
Fig.6.7 Amyloidosis. The splenic cut surfac e has a diffuse pale, waxy appearance . The spleen is more often involved in secondary. rather than primary, amyloidosis as may be seen in any long-standing infective or inflammatory con dition. Moderate splenomegaly commonly results . Fig.6.8 Myelofibrosis. This spleen is massively enlarged, weighing 3,175g, and is uniformly deep red in colour . The myeloproliferative disorders and chronic leukaemias are the commonest causes of massive splenomegaly. In the myeloproliferative cases this enlarge ment is due to the development of extramedullary haemopoiesi s (an integral part of the disease process, not secondary to marrow destruction), while in the leukaemias, there is extensive infiltration by neoplastic cells.
Fig.6.9 Chronic lymphatic leukaemia. Thi s SI'I" .. " ,', " enlarged and rather paler than normal. SmootillYI I'I ,I III,i. also visible at the hilum. Chronic lymphatic leuknll" " ,I" common and affects mainly the elderly with a prud! II"Ii'. 1 There is usually a massive circulating lymphocyl
Fig.6.10 Hodgkin's disease. The white pulp i ~, 0)'1 hili' replaced by innumerable , irregular, pale depo:;II:, 1111"11 the classical appearance of so· called 'salami' :;pl""1I 1i 1 disease, although almost any macroscopical chil li II Ii " I' Hodgkin 's lymphoma) may be seen . Splenic illvolv<'" ,, " up to 50% of cases of Hodgkin's disease and is "" " .1 . ,1 at staging laparotomy.
6 Lymphore ticular System
Fig.6.9 Chronic lymphatic leukaemia. This spleen is uniformly enlarged and rather paler than normal. Smooth lymphadenopathy is also visible at the hilum. Chronic lymphatic leukaemia is not un· common and affects mainly the elderly with predominance in men . There is usually a massive circulating lymphocytosis and generalised lymphadenopathy. Splenomegaly, as in chronic myeloid leukaemia , may be massive and the long· term prognosis is generally poor, although in the elderly life expectancy may not be affected.
Fig.6.ll Non-Hodgkin's lymphoma. This greatly en larged spleen is diffusely replaced by coarse nodular deposits of pale tumour . Splenic involvement is very common in any non-Hodgkin's lymphoma but particularly in the foll icular (Lukes and Collins) subtypes . Two points are worth remembering in any patient with lymphoma (1) the resultant splenomegaly may cause destruction of red cells, white cells or plalelets (hypersplenism) of itself , and (2) splenomegaly may be due to recu rrent or chronic coexistent infection.
Fig.6.l0 Hodgkin's disease. The white pulp is expanded and replaced by innumerable, irregular, pale deposits of tumour . This is the classical appearance of so·called 'salami' spleen in Hodgkin's disease, although almost any macroscopical distribution (as in non· Hodgkin's lymphoma) may be seen . Splenic involvement occurs in up to 50% of cases of Hodgkin's disease and is most often detected at staging laparotomy.
Fig.6.l2 Burkitt's lymphoma. Both kidneys and the liver are extensively replaced by mu ltiple large lymphomatous deposits . Burkitt's lymphoma, a specific sub type of non-Hodgkin's lymphoma, is typicall y a disease of children, most often seen in equatorial Africa. It is caused by Epstein·Barr virus and endemic malaria acts as a co·factor . It has a peculiar tendency to arise in the jaw, ovary, adrenal or kidney.
a
52
6 Lympho retic ular System Fig.6.13 Secondary carcinoma. Within the spleen are multiple, pale, um bilicated meta slases. Splenic metastases , surprisingly, are relatively un common, being macroscopically evident in only about 5% of cases of disseminated cancer examined at posl mortem. The most frequently responsible primary sites are the lung, breast, cutaneous malignant melanoma and ovary.
Fig.6.14 Tuberculous lymphadenopathy. These lymph nodes are densely adherent to one another and their cut surfaces show diffuse, irregular, caseous necrosis . Tuberculous infection in lymph nodes is usually seen in the regional nodes which drain the primary site,of infection, for example in the mediastinum, mesentery or neck. Atypical Mycobacteria may produce a similar picture . Healing often leads to dystrophic calcification, which may be an incidental radio logical finding .
53
Fig.6.15 Sarcoidosis. This mediastinal lymph node shows smooth, yellowish enlargement. Small amounts of anthracotic pigment are visible on the right. While sarcoidosis is the commonest cause of bilateral pulmonary hilar lymphadenopathy, lymph nodes at any site may be affected . Other organs that are commonly involved include skin (lupus pernio) , liver, muscle, eyes or bone .
Fig.6.16 Hodgkin 's disease. This group of lymph nodes each show rubbery, smooth enlargement but have remained discrete (cl. tuber culosis) . The cut surface is uniform and yellowish-white . Hodgkin's disease, a lymphoma of uncertain histogenesis, shows a predilection for males and has a peak incidence in the 2nd/3rd and 6th/7th decades . The Rye histological classification and Ann Arbor staging system correlate well with prognosis. There is epidemiological evidence (case-clustering) of an infective aetiology.
Fig.6.17 Non-Hodgkin's lymphoma. This IYII11.II '" III. enlarged and has a nodular, and in places almonl l, ,II" ! I surface . Non-Hodgkin's lymphoma is more COI11I"l" , 11111 disease and is particularly prevalent in the elduilv III.'" preceding autoimmune disease or iatrogenic 1111111111 11.· ,I some cases . There are several complex system: : "I l il t I classification, which are a source of confusion tl) ",, '"V, or Rappaport are probably the most used ,
Fig.6.18 Secondary carcinoma. These Iympll l it" I" ~ closely apposed to one another, are diffusely ropll l( I" expanded by pale , rather granular tumour. Metn:,I. ,II' the commonest source of neoplastic lymphadel1( I\ldli i particularly from carcinomas rather than sarCOII ll ll1, wi to haematogenous spread . Lymph node meta:itm,"', I worse prognosiS than if the tumour is confined tll II ', I II
6 Lymphoreticular Sy~t (" 111
Fig.6.17 Non-Hodgkin's lymphoma. This lymph node is smoothly enlarged and has a nodular, and in places almost follicular, cut surface . Non-Hodgkin's lymphoma is more common than Hodgkin's disease and is particularly prevalent in the elderly . There may be preceding autoimmune disease or iatrogenic immunosuppression in some cases . There are several comple x systems of histological classification , which are a source of confusion to many ; those of Kiel or Rappaport are probably the most used .
Fig.6.18 Secondary carcinoma. These lymph nodes, which are closely apposed to one another, are diffusely replaced and expanded by pale, rather granular tumour. Metastatic tumour is by far the commonest source of neoplastic lymphadenopathy, most particularly from carcinomas rather than sarcomas , which tend more to haematogenous spread . Lymph node metastases usually imply a worse prognosis than if the tumour is confined to its primary site.
Fig.6.19 Metastatic malignant melanoma. This lymph node contains a large, well circumscribed deposit of deeply-plgmented, focally necroti c tumour. A rim of uninvolved nodal tissue is visible on the right. Malignant melanoma is particularly prone to lymphatic invasion and lymph node involvement is a common finding at presentation . Fig.6.20 Thymoma. The thymus is greatly enlarged and is replaced by an encapsulated, multitobulated pinkish mass . The tumour lobules are separated by bands of fibrous tissue. Thymomas are un common, 8.rise most often in middle age and are slow growing, radio sensitive tumours with a generally good prognosis. Up to a third of cases may be associated with another systemic illness, in particular myaesthenia gravis or systemic lupus erythematosus .
54
7 Endocrine System
Fig.7.1 Pituitary adenoma. Arising in the pituitary fossa is a well circumscribed, rather haemorrhagic tumour. Small, non· functioning pit uitary adenomas are not uncommon. Symptomatic neoplasms, Wl'lich are less frequent, are most often composed 01 chromophobe cells (60%) or acidophils (30%): basophil adenomas are very rare. Such tumours may arise at any age and produce either local pre ssure effects (e.g. bitemporal hemianopia) or endocrine effects, Inost commonly due to the excessive secretion of prolactin or growth I~ ormone. Pituitary adenomas may also be seen in Type I Multiple endocrine Neoplasia Syndrome (Werner).
Fig.7.3 Thyroglossal cyst. This smooth, multilocular cyst contains clear, yellowish fluid and measures about 4 cm in maximum diameter. Such cysts represent vestigial remnants of the thyroglossal duct, along which the thyroid migrates in utero from the base of the tongue to its normal position. They may present at any age and are usually found in the midline of the neck, adjacent to the hyoid bone.
CLASSIFICATION OF THYROID ENLARGEMENT Graves' disease
~
HYPERTHYROID
Multinodular gOitre with toxic nodule Early Hashimoto's disease
r
Multinodular goitre Endemic colloid gOitre Adenoma Dyshormonogenesis EUTHYROID
primary Carcinoma
secondary
Lymphoma Fig.7.2 Craniopharyngioma. Arising in the region of the pituitary is a large, well circumscribed, tumour, with a variegated cut surface, whic h is compressing the optic chiasma anteriorly and the third ventricle superiorly. Craniopharyngiomas are traditionally held to nri se from Rathke's pouch and are usually suprasellar in location. r hey are slow-growing lesions which present most often in childhood, llsually due to pressure on the pituitary or optic tracts. They very commonly undergo cystic change or calcification.
55
De Quervain's thyroiditis End-stage multinodular goitre HYPOTHYROID
Chronic Hashimoto's disease Endemic cretinism
Fig.7.4 Classification of thyroid enlargement.
Fig.7.S Multinodular goitre. The thyroid is distort l\l II IV III nodules of varying size, some of which contain collr'll l Wi lli, have undergone calcification and cystic chang e. I ri ll ' " " " I' apparent, particularly in the left upper pole. Multlr1n
Fig.7.6 Colloid goll.. thyroid is ma ssiVl 'lv ," ,I, multiple diffuse I\nd lllllil which are 'menly' 'I 1. 11 11 while others aPI" '.11 , V', colloid-l illed. WI,I"'II" appearance rn: Iy 11'1,, ," early stages of :.'1 111111 1' ,1 toxic goitre due III ,,, IV ' prior to the devul(){l" II JI ' volutional or c! eCjll""1. 01 1 changes), diffLlsl ' I' ,II, i,i are most oft en 01l ilol lIll iodine deficiency (I hil i " the drinking watql ) II II'. commonest in 11111111 rI .ri ll areas (e.g. th8 1\11'" I II Himalayas) alld W; II. " I, a problem alon!) 1111' I " I> this country (, 001I,v' II 1>1'
7 Endocrint: Syslt'11
IOssal cyst. This smooth, multilocular cyst contains 1IIIIIrI nnd measures about 4 cm in ma xim um i I V'JIt1 represenl vestig ial remnanl s of lhe thyroglossal I ' I1111 0 Ihyroid migrates in utero from the base of the position . They may presenl at any age and are III " , •'lIdline of the neck. adjacenl to the hyoid bone .
"." .1
ATION OF THYROID ENLARGEMENT
Graves' disease ItY HOID
Multinodular goitre with toxic nodule Early Hashimoto's disease Multinodular goitre Endemic colloid goitre Adenoma Dyshormonogenesis
U II )
primary Carcinoma
secondary
Lymphoma De Quervain's thyroiditis End-stage multinodular goitre I Ifjln
Chronic Hashimoto's disease Endemic cretinism
11(''111,1011 of thyroid enlargement.
Fig.7.S Multinodular goitre. The thyroid is distorted by multiple nodules of varying size, some of which contain colloid while others have undergone ca lcification and cys tic c hange . Fibrosi s is also apparent, particularly in the left upper pole. Multinodular goitres, wh ich are quite common, represent the end stage of a diHuse non-toxic goitre the latter may be endemic, due to iodine deficiency, or sporadic, due to environmental or genetic factors. Patients with multinodular goitre are usually euthyroid but may sometimes develop autonomous toxic nodules . Fig.7.6 Colloid goitre. The thyroid is maSSively enlarged by mulliple diffuse nodules, some of which are 'meaty' in appearance while others appear cystic or colloid -filled . While this appearance may represent the early stages of a diffuse non toxic goi tre due to any cause (i e prior to the development of in volu tional or degenerat ive chang es), diffuse colloid goitres are most often endemic due to iodine deficiency (particularly in the drinking water). This is commonest in mountainous areas (e.g. the Alps or Himalayas) and was at one time a problem along the Pennines in this country ('Derbyshire neck')
Fig.7.7 Graves' disease. The thyroid is diffusely and smoothly enlarged; the cut surface ha s a 'beefy' appearance. Graves' disease (diffuse toxic goitre) is commonest in young women and is an auto immune disease, the most important autoantibodies found being those thai mimic the actic;1 01 TSH. Affected individuals are thyrotoxic and may show associated ophthalmopathy, pretibial myxoedema and enlargement of lymphoid tissues. Thi s condition is c losely relate d to Hashimoto's disease and , if treated surgically, some pa tient s may develop hypothyroidism.
Fig.7.S Hashimoto's disease. This thy roid is also diffusely and smoothly enlarged but the cu t surface has a pale appearance (likened to normal pancreas) . Hashimoto's disease is another au to immune disease which shows a very marked predilection for young adult females, and results in autoantibody-mediated destruction of follicular cells. Patient s may also have coexistent perniciOUS anaemia, Sjog ren' s syndrome or Addison's disease . At teast50% eventually become hypothyroid .
56
7 Endocrine System Fig.7.9 Primary myxoedema . This thyroid , shown in situ with the trachea, is markedly shrunken , pale and fibrotic . Primary hypothyroidism in adults is usually due to chronic auto· immune thyroidit is and probably represent s end·stage Hashimoto's disease . As such , middle·aged females, some· times with ot her autoimmune conditions, are pred ominantly affected. Other causes of clinical hypolhyroidism include an end · stage goitre , previous thyroidec· tomy or irradiation and , rarely , dyshormonogene sis.
Fig.7.11 Follicular carcinoma. Within this lobe of the thyroid , distorted by a multinodular goitre, a pale infil· trative neoplasm is visible in the lower pole . Follicul ar car cinoma (25% of thyroid malig nancies) is rather commone r in females and tends to occur in middle age Occasionally, it can be distingu·,shed from a follicular adenoma only by microscopic evidence of vascular invasion . This tumour lend s to local infiltra tion and vascular spread (in contrast to the lymphatic sp read of papillary ca rcinoma) with a 5-year surviva l of about 60% .
Fig.7.13 Medullary carcinoma. The cut suil:u :" "III,. serial sec tions through the right lobe of thi s lilY" III I · ,I" '. reasonably circumscribed tumours of va rying : 01/ " MH. I cinoma, which is derived from parafollicular c: ill ,I I " il!' cells, accounts for about 10 % of thy roid cance r', 1111 10 , (usually in middle age ) or familial (affecting yCII IIIII' 'I "" I often multifoca l), forming part of the Typ e II MI ilt'jlll ' I II I Neoplasia Syndrome (Sipple) , 5-year·survivall:. il t I( 11 " •
Fig.7.12 Anaplastic car cinoma. This large, pale and
Fig.7.10 Papillary carcinoma. Arising in the righ t lobe of an o th er· wise normallhyroid is a pale, irregular neoplasm: a lymph node infiltrated by metastatic tumour is adherent to the lower pole . Papillary carcinoma accounts for about 60% of malignant thyroid neoplasms . It arises most often in young adults and shows a predilection for females . It is sometimes multicentric in origin and occasionally presents with an adjacent lymph node metastasis (known previously as the ·Iateral aberrant thyroid'). The overaIl5·year-s urvival is about 90% .
57
focally haemorrhagic tumour has replaced most of the thyroid and is involving adjacent lymph nodes and the tracheal wall by direct extension, There is an associated florid tracheitis , Ana plastic thyroid carcinoma, which is relatively uncommon , is vi rt ually confined to the elderly , It is a rapidly progressive and lethal tumour, which tends to very extensive local invasion (otten with tracheal stenosis) and is usually fatal within a year of diagnosis.
Fig.7.14 Lymphoma. Arising within and enl:II \l IIII III" the thyroid is a diffuse pale neoplasm ShOWlllP 11111 1111." Primary lymph oma of the thyroid is uncommOl' ,II" I " . 1 elderly females, often with evidence of pre ux,,;I, '1111 I.,' disease . Such tumou rs are usually non-Hod~k u,", II " YI ' any systemica lly dissem inated lymphoma m:ly "'V' .I v" I secondarily . In general the prognosis is poor
7 Endoc rine Sy~ ... n l
Fig.7.13 Medullary carcinoma. The cut surface of the lelt lobe and serial sections through the right lobe 01 this thyrOid show mu ltiple . reasonably circumscribed tumours of varying size . Medullary car cinoma. which is derived Irom parafollicular calcitonin-secreting cells. accounts lor about 10 % 01 th yroid cance rs . It may be sporad ic (usually in middle age) or familial (affec ting younger individuals and often multifocal). forming part of the Type II Multiple End ocrine Neoplasia Syndrome (Sipple) 5-year-survival is about 50% .
Fig_7_1 4 Lymphoma. Arising within and enlarging the left lobe of the thyroid is a diffuse pale neoplasm showing multifocal necrosis . Primary lymphoma of the thyroid is uncommon and is typically seen in elderly females . often with evidence of pre-existent Hashimoto'S disease . Such tumours are usually non-Hodgkin's in type. although any systemically disseminated lymphoma may involve the thyroid secondarily . In general the prognosis is poor.
Fig.7.15 Parathyroid hyperplasia_ Each of these four parathyroid glands is marked ly. although rather unequally. enlarged; all have a nodular appearance. Parath yroid hyperplasia is relatively uncommon. being responsible lor only about 10% of cases of hyp er parathyroidism . It may be a primary idiopathic lesion , sometimes forming part 01 a Multiple Endocrine Neoplasia Synd rome. or it may be secondary to chronic renal failure. In long-standing cases of the latter type, autonomous true adenomas sometimes develop . Fig.7.16 Parathyroid adenoma. Beneath the lower pole of the right lobe of the thyroid. viewed anteriorly, is a soli tary. large. browni sh parathyroid tumour. Parathyroid adenomas are responsible for at teast 75% of cases of hyper parathyroidism and are commonest in middle age. alfecting predominantly femates They are usually solitary and sometimes arise in long standing secondary hyper parathyroidism. Elevated levels of parathormone, which more rarely are due to parathyroid carcinoma or ectopic secretion by a heterotopiC malignancy, lead to nephrocalcinosis and osteitis fibrosa cystic a (see Chapter 12) .
5t1
7 Endocrine System Fig.7.17 Waterhouse Friderichsen syndrome. The adrenal gland (top) , the outline of which is just visible, has been totally destroyed by extensive haemor· rhage . Below, the feet of a young boy show the typical petechial rash, with cyanosis and gan· grene. Waterhouse Friderich sen syndrome refers solely to the occurrence of adrenal haemor rhage associated with a severe seplicaemic illness . It is classically seen in children or young adults, most often with meningococcal septicaemia The ad renal haemor rhage, which is usually fatal , is thought to result from a combination of endotoxin induced ce ll damage, 'stress' and a degree of disseminated intra vascular coagulation .
\,,1
Fig.7.18 Primary Addison 's disease. This adrenal gland. seen in cross-section. is markedly shrunken and thinned, measuring less than 2 cm in lenglh. Addi son's disease, or ch roni c adrenocortical insufficiency, is most often due to idiopathic autoimmune destrUClion. analagous to primary myxoedema. More Ihan 90% of adrenocortical tissue has to be lost before the typical symptoms or electrolyte disturbances result.
Fig.7.19 Addison's disease due to tuberculosis. The cortex and medulla of this adrenal show ex tensive caseous nec rosis and the surroundi ng capsule has undergone dystrophic calcification. Prior to the recognition of autoimmune disease, TB was regarded as Ihe commonesl identifiable cause of Addison's disease , although this occurrence is now rare in the Western World . However, it is typica lly a complication of pre-existent pulmonary infection . Other causes of Addison's disease include amyloidosis. melastatic carc inoma. haemochromatosis and histoplasmosis.
S9
Fig.7.20 Ad roll"" 1 nodular hYPllqlhull cortex ClIIIII', li d II ,, ' • which lIiI~; 110"" 1II bisecled, I plll " 'II'. '" brigll t y( )IIClW 11111 1111 1 of which is VI'IIl,I, 1 .01 I Such no(llIl. " " VI" 'I idiopatllic ,,",1, '. I'" I more COil II II{)II 11 ... 11 I adenoma 1111 ' I,"" II however,III,c'II I. II" nodules ::Ir!' IVl lII ..II'.' funclionill!j ; 11 111, I" I · to any elld,)( I li lt· ,I' ll This app orll I 11 11 , ," associal od Will, I " ' ii i tension , all 1111' " 1,11,11 phenomCllC111
Fig.7.21 Adrenocortical adenoma. On Ihe- Iell , I I ' ,11"" circumscribed lumour projects from the COrlic; 11 ,11)( I,,, , adrenal. The same gland in cross-seclion (riglll) ',1" 'w' brownish lesion with marked atrophy of the aCliftt , ' I II , , II I solitary functioning adenomas. as opposed Iu c( li l li , II", above) . are relatively uncommon but may re silli '" ( '" !I I syndrome with consequent pituitary suppresslol' (, I ' . I,d, hyperaldosteronism (Conn's syndrome) .
7 Endocrine
Fig.7.20 Adrenocortical nodular hyperplasia. The cortex of this adrenal gland. which has been partially bisected , contains multiple bright . yellow nodules, the largest of which is visible at the apex. Such nodular hyperplasia is idiopathic and is probably far more common than a solitary adenoma . The true incidence is, however, uncertain since these nodules are typically non· functioning and do not give rise to any endocrine disturbance. This appearance is sometimes associated with benign hyper· tension. an unexplained phenomenon
Fig,7.21 Adrenocortical adenoma. On the left , a smooth, well circumscribed tumour projects from the cortical surface of an adrenal. The same gland in cross-section (right) shows a solitary brownish lesion with marked atrophy of the adjacent cortex. Such solitary functioning adenomas , as opposed to cortica l nodules (see above), are relatively uncommon but may result in Cushing's syndrome with consequent pituitary suppression (as here) or in hyperaldosteronism (Conn's syndrome)
~y:-' l llli
Fig.7.22 Adrenocortical carcinoma. This adrenal gland , measuring 20cm in maximum diameter, is massively enlarged by a multinodular tumour showing extensive cystic change and haemor rhage. Adrenocortical carcinoma is rare but may occur at any age. These tumours are typically non-functioning and therefore otten attain a great size prior to presentation The presence of metastases is far more reliable than the histological appearances in distinguishing malignant lesions but, in general, these latter tend to rapid and extensive haematogenous dissemination. Fig.7.23 Meta static carcinoma. This partially bisected adrenal gland is irregularly distorted by numerous nodules of pale secondary tumour, some of which show necrosis or haemorrhage, While any malignant tumour may metas tasise to the adrenal, by far the commonest to do so is carcinoma of the bronchus, followed by breast carcinoma and malignant melanoma . Such spread is usually bilateral and may occasionally give rise to Addison's disease (see Figs. 7.18&7 .19)
60
7 Endocrine System
Fig.7.24 Adrenal lymphoma. Th is adrenal gland is totally repl aced by an irregular mass of pale yel lowish -pi nk Jissue. Primary lymphoma of the adrenal gland , whi ch is usually non-Hodgkin's in type, is extremely rare, Even secondary involvement by any histological type undergoin g systemic dissemination is very uncommon,
Fig.7.25 Phaeochromocytoma. The adrenal medulla is g rossly expanded and repla ced by a tan-coloured, rather vasc ular tumour showing foci of haemorrhage, The attenuated cortex is visible as a yellow rim of tissue. Phaeochromocytomas arise from chromaffin cel ts, typically in young adults, and ma y be associated wit h neuro fibromatosis or Type II Multiple Endocrine Neoplasia Synd rome, While Ihe vast majority are benign, they secrete excessive amoun ts of catecholam ines , giving rise to paroxysmal hypertension , They are not uncommonly bilateral.
61
Fig.7.26 Neuroblastoma. Re placing the left adrenal (top right) is a large, multinodular haemorrh agic mass, Deposits of metastati c tumour are vis ible in this child's skull and femur. Neuroblastomas arise from non ch romaffin cells in the adrenal medulla (or sympathetic chain) and are seen almost solely in young c hildren. They are highly malignant lesions, which occasionall y secrete catech ola min es; modern modes of treat ment have led to an im p roved survival rate. Interestingly, lesions in the right adrenal metastasise more ofte n to the liver, in contrast tothe case here.
Fig.7.27 Chemodectoma . This specimen comprises a we ll circumscribed , b rowni sh, vasc ular mass showing areas of haemor rhage. Chemodectom as are the commones t neuroendocrine tu mours of the extra·adrenal paraganglionic system and may be seen at any age They most often arise in th e ca roti d body and are commo ner in populations living at high altitude (p robabl y as a co nsequence of prolonged hypoxia) . Similar lesions arising in the temporal bone or at the base o lthe skull are known as glomus jugulare tumou rs. A varia b le number behave in a malignant fashion
Fig.8.1 Fetal renal lobulation. These are kit III! 'Y' , II , III 1 infant note the marked corlicallobulation. Ti ll, ', " 1'1" " II , I normal but is usually no longer apparent by (lI lt' Y",,, , d ever, lobula ti on, if only partial, sometimes PO",,,,I', 1111". is important to recog nise that such a finding i:: ," II" I', III im portance .
Fig.8.2 Horseshoe kidney. The kidney s fH(! III""tI ,.111 poles and both renal hila lie anteriorly . Th e d 1111 III; , , " " " whi ch joins Ihe two kidneys la yover the aorlilill VI V" HI 'I oc curs in at least 1 in 250 individuals and resllll' , 1/"" , I II embryological ascent of nephrogenic lissue: , 1(Jlluw", 1I" Coexistent anomalies of th e ureters or renal VI",',' ,I' , .11,11 seen and may predispose to urinary infeclinll (il "I,',I""
8 Urinary Syste m Fig.7.26 Neuroblastoma. Replacing the left adrenal (top right) is a large. multinodular haemorrhagic mass. Deposits of metastatic tumour are visible in this child's skull and femur . Neuroblastomas arise from non chromaHin cells in the adrenal medulla (or sympathetic chain) and are seen almost solely in young children. They are highly malignant lesions, which occasionally secrete catechola mines; modern modes of treat ment have led to an improved survival rate . Interestingly. lesions in the right adrenal metastasise more often to the liver, in contrast to the case here .
111odoctoma. This specimen comprises a well n.' 'w n l ~ h. vascular mass showing areas of haemor I", h Irn:,s are the commonest neuroendoCrine ! II.' .It/renal paraganglionic system and may be " I I'I 'Y 11'10s1 often arise in the carotid body and are Ii 'I (l 1 ~. IN)lIS living at high altitude (probably as a
,11'" ,low led hypoxia) Similar lesions arising in the
. 'I ." 11 11>base of the skull are known as glomus
, ," 1\ v,,"nl')le number behave In a malignant fashion .
Fi,.8.1 Fetal renal fobulation. These are kidneys from a stillborn infwl : note the marked cortical lobulation . This appearance is enlirely nOmal but is usually no longer apparent by one year of age How e~r, lobulation. if only partia l. sometimes persists into adult life and it is rnportant to recognise that such a finding is of no pathological iiTl)ortance.
I I
Fi\.8.2 Horseshoe kidney. The kidneys are fused at their lower pees and bOlh renal hila lie anteriorly. The isthmus of renal tissue wt.c h joins the Iwo kidneys layover the aorta in vivo. Renal fusion OO;urs in at least 1 in 250 individuals and results from partial failure of enbryological ascent of nephrogenic tissue, followed by malrotation. Cr.existent anomalies of the ureters or renal vessels are often also se,n and may predispose to urinary infection or obstruction.
Fig.8.3 'Infantile' polycystic disease. The renal parenChyma of this adolescent's kidney is completely replaced by thin wa lled cysts Infantile polyc ystic disea se ma y take a va riety of forms . Classically , it has an autosomal recessive Inheritance and take s a fatal course in infancy. In such cases the kidneys appear normal exter nally but show numerous small. radially arranged cysts on sectioning. There IS always associated congenital hepatic fibrosis ; in some cases (as here) the renal appearances and clinical duration may be ve ry va riable Fig.8.4 Adult polycystic disease. This kidney has been bisected th rough the hilum to show extensive parenchymal replacement by cysts of varying size, into some of which haemorrhage has occured. Adult poly cystic d isease is an autosomal dominant inherited condition ; patients typically presenl in middle age and chronic renal failure usually supervenes there after. Associated hepatiC or pan creatic cysts and cerebral berry aneurysms may also be found.
62
8 Urinary System Fig.a.S Medullary sponge kidney. The cut surface of this kidney shows multiple, smooth walled cysts which are confined to the renal papillae. This condition, thought to be due to develop mentally anomalous collecting ducts, affects males more than females and is usually detected in the 5th or 61h decades. Calculi often develop within the cysts and, in combination with recurrent urinary infection, may lead to impaired renal function .
Fig.a.6 Renal dysplasia. Much, but not all , of this kidney is rep laced by coarse, irregular cysts separated by broad bands of fibrous tissue. Renal dysplasia, which represents failure of nephrogenic dif ferentiation, may affect one or both kidneys and may involve either the whole, or only part, of the kidney. Often other developmental anomalies, usually obstructive in nature , are present elsewhere in the urinary tract.
63
Fig.a.7 Simple renal cyst. Arising from the cortex of the lower pole of this kidney is a large, thin-walled cyst. Simple ,enal cysts are extremely common, particularly with advancing age, and are thought to represent the local effect of previous ischaemia or obs truction. They are usually solitary, are typically located in the cortex and are most often only about 1 cm in diameter, the exam ple here being unusually large.
Fig.a.a Acute pyelonephritis. This hemisected kidney shows intense con gestion and in numerable , radially arranged yellow areas of suppuration and abscess forma tion, especially in the medulla. Acute pyelonephritis is not uncommon and is usually a con sequence of ascending Gram negative infection. Common predis posing causes include urinary obs truction, diabetes mellitus and preg nancy . In general, females are most often affected , probably as a con sequence of peri urethral contami nation by faecal organisms.
Fig.a.g Renal tuberculosis. The parenchYI' "" d 'III kidney shows numerous confluent foci of CW,IICiIl' , ' ,, ' , also marked calyceal invotvement with dllllt.liI"" , ' /'VII called 'pyonephrosis' (despite the absellc(.! ,,11 111' 1) I culosis is often bilateral, is commonest in a dilli I fllIl! due to haematogenous spread from prima,y II d, ,' III " While remaining endemic in some part s 0 11111' Wi "I i I now uncommon in Caucasians. I III
I
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8 Urinary System
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Fig,B,9 Renal tuberculosis, The parenchyma of this bisected kidney shows numerous confluent foci of caseous necrosis; there is also marked calyceal involvement with dilatation, giving rise to so called 'pyonephrosis' (despite the absence of pus). Renal tuber culosis is often bilateral, is commonest in adult males and is usually due to haematogenous spread from primary infection elsewhere. While remaining endemic in some parts of the world, this infection is now uncommon in Caucasians.
te i ~is_
This lidney e con
{l
pdially
Fig,B.10 Chronic interstitial nephritis. The capsular surface of this kidney shows coarse , irregular scarring and the whole organ is rather shrunken. Chronic interstitial nepl1ritis is the term used to describe chron ic parenchymal inflammation and atrophy , which may be a consequence of various conditions including chronic suppurative pyelonephritis , long standing ischaemia or obstruction and analgesic nephropathy.
lOW
·)uration forma lly in the Ite s is not ndis
ram ction . dis
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Fig,B,11 Renal calculus, Lying within the renal pelvis is an irregular, ovoid stone . Surprisingly , there is no evidence of hydronephrosis. Calculi in the urinary tract are common, are seen most frequently in the kidney and usually present in adult hood . Predisposing causes include urinary obstruction, an elevated urinary concentration of the relevant constituent or altered urinary pH facilitating crystal precipitation .
Fig,B,12 Staghorn renal calculus. This stone has a branched appea rance (resembling the antlers of a stag) and formed an accurate cast of the pelvicalyceal system and upper ureter from which it was removed. Staghorn calculi are typically composed of calcium phosphate (the commonest constituent of renal stones) but may also be made up of 'triple' phosphate or cystine . Compli cations of urinary lithiasis include obs truction, infection and haematuria.
64
8 Urinary System
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Fig.8.l3 Renal infarct. Much of the renat cortex shows marked pallor, allhough the lower pole is spared. The margins of the infarct are hyperaemic. This is an unusually large renal infarct, small wedge· shaped lesions being more common. Infarc tion of the kidney is nearly always due to arterial occlusion by embolism or thrombosis. Emboli are usually cardiac in origin and, if derived from the vegetations of bacterial endocarditis, may give rise 10 septic infarcts with abscess formation . Fig.8.l4 Renal corticaf necrosis. The entire renal cortex is pale and necrotic, while the corticomedullary junction is congested . Renal cortical necrosis is usually a conse quence of disseminated intra vascular coagulation , as may occur in antepartum placental haemorrhage, septicaemic illnesses or severe trauma. Acute rena l failure rapidly super venes and the prognosis is generally poor Multifocal cortical necrosis may also be seen in the haemolytic·uraemlc syndrome.
65
lli 'V' 01 1'I ii ",11 " ' 1 11\
Fig.8.l5 Renal vein thrombosis. The entire kidney is pale and the main renal vein is completely occluded by organised thrombus. While renal vein thrombosis may be acute in neonates (giving rise to red infarction), in older individuals the onset is usually gradual , resulting in oedema and tubular atrophy. In the latter group, throm· bosis is usually secondary to chronic glomerulonephritis or amyloidosis and the outcome is most otten fatal.
fll ll'11 Iii
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Fig.8.l6 Acute transplant rejection . The renal cortex is pale, swollen and shows small petechial haemorrhages. The medulla is very intensely congested. This is a classical example of acute rejection which usually occurs within a year of transplan· tation and is most often due to the development of recipient anti-graft antibodies . Rejec tion may also be hyperacute, due to pre-existent sensiti sat ion to donor antigens, or chronic (see Fig.B.17).
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8 Urinary
Fig .8.16 Acute transplant rejection. The renal cortex is pale, swollen and shows small petechial haemorrhages. The medulla is ve ry intensely congested . This is a c lassical example of acute rejection which usually occurs within a yea r o f transplan tation and is most often due to the development of recipient anti-graft antibodies. Rejec tion may also be hyperacute , due to pre-existent sensiti sat ion to donor antigens, or chronic (see Fig.B.17).
I
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Fig.8.17 Chronic transplant rejection. This kidney shows a non specific, congested and rather mottled appearance Chronic rejection of a renal transplant tends to occur several months, or even years, after grafting and is often characterised by the asymptomatic development of either hypertension or the nephrotic syndrome. It is thought to be due to chronic low-grade arterial damage (due to deposition of microthrombi), combined with complex- mediated glomerulonephritiS and tubular atrophy.
Fig.8.19 Acute proliferative glomerulonephritis_ Thi s bisected kidney is rather swollen and there are petechial haemorrhages in the cortex Acute proliferative glomerulonephritis is fairly uncommon, occurs predominantly in children and usually follow s a group A streptococcal upper respiratory infection. It is thought to be due to deposition of circulating immune complexes and often gives rise to the nephrotic syndrome. In the majorit y of cases spontaneous recovery occurs.
Fig.8.18 Essential hypertension. This kidney is slightly shrunken and the capsular surface shows fine granular sca rring . along with several small si mple cysts . Renal chang es in benign hypertension are largely microscopic and are principall y isc haemic in nature. There is usually little if any impairment of renal function but up to 5% of patients devel op malignant hypertension with subsequent renal failure.
Fig.8.20 Mem branous glomerulo nephritis. This kidney has been sectioned to show extreme cortical pallor, oedema and blurring of the corticomedullary junction . Mem bra nous glomerulo nephritis is an idio pathiC cond ition, due to the deposi tion of circulating immune comptexes. which typicall y presents in adult hOOd . Up to 70% of patients develop chronic renal failure .
66
8 Urinary System - - - -- - - - -
-- -
CAUSES OF NEPHROTIC SYNDROME minimal change membranous Glomerulo nephritis PRIMARY RENAL DISEASE
membrano proliferative focal segmental glomerulo sclerosis
Congenital
Alport's syndrome
Renal vein thrombosis Diabetes mellitus Amyloidosis Systemic lupus erythematosus SECONDARY RENAL DISEASE
Drugs
penicillamine hepatitis B
Infections malaria lymphoma Malignancy
Fig.8.21
67
Causes of nephrotic syndrome.
bronchial carcinoma
Fig.8.22 Chronic glomerulonephritis. Thi s kidney is shrunken and shows severe granular scarring of the cort ical surface. Chronic glomerulonephritis is the non-specific end-stage form of many primary glomerular diseases. However, in a proportion of cases , the prima ry initi ating episode has passed unnoticed. Chronic renal failure always develops and , without dialysis or transpl antation, death is invariable . Fig.8.23 Chronic glomerulonephritis with secondary cystic change. In addition to being markedly shrunken and scarred, the renal parenchyma contains innumer able small cysts. This app earance is now well recogn ised in patients who have been maintained on long-term dialysis for chronic renal failure . It is thought to be a secondary phenomenon, perhaps related to renal tubular obstruction .
Fig.8.24 Renal papillary necrosis. In both "l l ir ,I'Y" III ' papillae show greyish necrosis; on the right. :;( >1 111' , dil l' have sloughed off. Renal papillary necrosis n!(J: ,1 "II, '" '' complication of diabetes mellitus , analge SIC CIIIII' .I ' lill I anaemia or urinary obstruc tio n with sup erackl ('( I >I II" , II, va scular damage is thought to be respon Slblt' II, I'" ,. Ii I colic or oliguria may result.
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8 Urinary System
mlc glomerulonephritis. This kidney is shrunken and If .1l1ular scarring of th e co rtical surface. Chronic 11,11', IS the non-specific end-stage form of many jlll.1I diseases . However. in a proportion of cases. the 11 1 "pisode has passed unnoticed . Chronic renal Ilw(J lops and. without dialysis or transplantation, deat h
Fig.8.23 Chronic glomerulonephritis with secondary cystic change. In addition to being markedl y shrunken and scarred, the renal parenchyma contains innumer able small cysts. Thi s appearance is now well recognised in patients who have been maintained on long-term dial ysis for chronic renal failure . It is th ough t to be a second ary phenomenon, perhaps related to renal tubular obstruction.
Fig.8.24 Renal papillary necrosis. In both kidneys the renal papillae show greyish necros is; on the right . some of th e papillae have sloughed off Renal papillary necrosis most often occurs as a complica tion of diabetes mellitus , analgesic abuse. sickle cell anaemia or urinary obstruction with superadde d infec tion. Micro vas cula r damage is thought to be responsible in most cases ; renal colic or oliguria may result. Fig.8.25 Hydro nephrosis. The pelvicalyceal system is grossly dilated . with marked atrophy of the cortex and medulla. There is florid pyelitis. Hydronephrosis result s from distal urinary obstruction. causes of which inc lude pro static enlargement. pelvic or ureteric tumours , ure teric calcu li . neuromusc ular defects and retro peritoneal fibrosis. Thi s condi tion IS often complicated by urin ary infection but renal function is impaired only if the obstruction is bilateral.
Fig.8.26 Renal amyloidosis. The renal parenchyma shows waxy pallor with blurring of the cortico medull ary junc tion The ki d ney is more often involved in secondary than primary or myeloma-associated amyloidosis . Amyloid is a fibrillary protein. arranged in 13 pleated sheets, which is derived from serum amyl oid A protein in secondary cases and from immunog lobulin light chain s in the primary or mye loma associated group. Renal involvement is bilateral and usually result s in proteinuria and chron ic renal failure.
Fig.8.27 Renal cortical carcinoma. Arising in the upper pole of this kidne y is a large, lobulated mass; the cut sur face has a typically variegated appea rance. being yellowish wi th foci of haemorrhage and nec rosis . Renal cortical ca rci noma (clear cell car cinoma , hype r nephroma, Grawitz tumour) arises most often in the 6th and 7th decades. affects predominantly me n and is deri ved from tubular epilhelium . Spread is largely haematogenous, including direct exten sion within the renal vein. 5-year surviva l is abou t 40 % .
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8 Urinary System Fig.8.28 Pelvic transitional cell carcinoma (TCC). Above, a small papillary tumour is seen arising in the renal pelvis; below, a much larger, more solid tumour has filled the renal pelvis and caused secondary hydro nephrosis. TCC of the renal pelvis is not uncommon, arises most often in the 6th and 7th decades and may be associated with similar lesions in the bladder or ureter. Workers in the ani line dye and rubber industries, along with cigarette smokers, are at increased risk. OveraI15-year survival is about 40%, solid tumours carrying a worse prognosis than papillary. The development of multi focal neo plasms along the length of the urinary tract is now regarded as a 'field change' effect rather than metastatic 'seeding' from a proximal lesion.
69
pi lII'I 1111 jill ' 11,,11 1111 1>1 11" ,
l,yllll)I" '1 II I1 ,V I ,, " 1'1I
11i 11
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Fig.8.29 Nephroblastoma. This kidney is largely replaced by a large, well circumscribed tumour. The cut surface is pale with cystic, haemorrhagic and myxoid foci. Nephroblastoma (Wi 1m's tumour) is one of the commonest malignancies in infancy, affects predominantly males and almost invariably presents by the age of 7 years It may occasionally be bilateral or associated with corporal hemihyper trophy or aniridia. With modern multimodal therapy, the prognosis is extremely good. Fig.8.30 Ureteric duplication. Ema nating from this renal pelvis are two separate ureters. The kidney is other wise normal. Congenital anomalies of the urinary tract are common and may be associated with. genital tract malfor mations. Up to 3% of the population may have accessory ureters, although the bifurcation is usually distal to the renal pelvis.
11/111111 " '1 Vllklpll l! til IrM 1111 1
Fig.8.32 Urourlll Projectinq 11<," ,11" , ' I face of the 111 1,1, 11 III' small, thi n,wlIlI,,>!. ,' V structures, I jllll"l1l1 repres en ls
8 Urinary System Fig.8.31 Pel vi ureteric junction obstruction. This kidney shows marked hydrone phrosis with gross pelvic dilatation In vivo, two aberrant arteries (bottom right) were com pressing the proximal end of the ureter (bottom left). Obstruction at the pelvi-ureteric junction is an important cause of hydronephrosis and may be due to idio pathic neuro muscular incoordi nation or a de velopmental anomaly of the renal vessels or ureter. Fig.8.32 Ureteritis cystica. Projecting from the urothelial sur face of the ureter are multiple, small, thin-walled, cystic structures. Ureteritis cystica represent s a result of long standing chronic inflammation, of whatever cause, and is entirely comparable to cystitis cystica in the bladder. The cysts are dilated von Brunn's nests, which are localised down growths of urothelium often seen in chronic inflammation. Occasionally ureteritis cystica may give rise to ureteric obstruction.
Fig.8.33 Ureteric transitional cell carcinoma (TCC). Emanating from the posterior wall of this thickened ureter is a rounded solid tumour (top); a cross-section through a separate lesion demonstrates well the typical papillary nature of a Tee (bottom). The pathology of ureteric Tee is much the same as that in the renal pelvis (see Fig .8.28). Multifocal Tee in the urinary tract is common, and is thought to represent a 'field change' effect. It is said that the thin ness of the ureteric wall predisposes to early deep invasion of these tumours and hence to rapid lymphatic spread. Generally, the prognosis of these tumours is worse than that of their counterparts in the bladder.
70
8 Urinary System Fig.8.34 Bladder diverticulum. Projecting from the fundus of the bladder is a thin-walled diverticular sac (above); the bladder itself is trabeculated and markedly congested. Bladder diverticula are usually a consequence of outflow tract obstruction and are therefore commonest in elderly males with prostatic hyper trophy. Because urine stagnates within such diverticula, secondary infection and stone formation are common. The development of carcinoma is also a well recognised complication .
Fig.8.35 Vesical papillary transitional cell carcinoma (TCC). Projecting from the urothelial surface is a small papillary tumour composed of innumerable frond-like excrescences. In the urinary tract, the bladder is the commonest site of origin of TCC. In addition to the aetiological factors mentioned in Fig.8.28, other predisposing causes include schistosomiasis, bladder diverticula and exstrophy. Multifocality is common and the overall mortality is about 50%, many deaths resulting from the complications of obstruction or infection rather than from metastases .
71
Fig.8.36 Vesical solid transitional cell carcinoma (TCC). This bladder has been opened to show gross urothelial distortion by a multi lobulated , predominantly solid, pale neoplasm . A papillary area is visible just above the urethral orifice. Solid vesical TCC is less common than the papillary variant, although the two patterns may be mi xed . Solid lesions, which are usually poorly differentiated histo logically, carry a worse prognosis.
Fig.9.1 Corpus luteum. Within the ovary is d Willi I III " nodule, measuring 1.5 cm in diameter. Th e 0\111 ,,1111, I' , I in colour , while centrally it is composed of IOOb!.; II, ,,11 '11 I) within which is a small cavity. The ovarian foil ic /t , 1' ,11 "I, corpus luteum after ovulation, during the COIIf ~j ' "I wI II, rhage into the central cavity is invariable . The YI 'lIuw '''' the luteinised granulosa and theca cell layers , WII Il .11,,, I secrete progesterone and, to a lesser extent, CJ( ",II I "1111 paration for implantation . If fertilisation does 11(11 " I 'I II I luteum involutes.
Fig.8.37 Urethral valve.. A flap of redundant epith elium is visible in the prostatic urethra (marked with a green probe); the bladder is trabecu lated and congested and there is marked bilateral hydroureter and hydronephrosis. This congenital mal formation is virtually confined to males and is one of the commonest causes of urinary obs truction in infancy. Age at presentation is dependent upon the degree of obstruction.
Fig.9.2 Corpora albicantia. This atrophic OVIllY 1""1 ' 11 woman contains multiple small, yellowish-whito 111 " 1,,1, ,, albicantia represent corpora lutea which have 1111< 1' " 1I'" logical involution, being replaced largely by llynl" 1111111I, such, they persist after the menopause when II II ' Y I " II , " conspicuous as a consequence of ovarian atr0pliV
9 Female Reproductive System
Fig.9.1 Corpus luteum. Within the ovary is a well circumscribed nodule, measuring 1.5 cm in diameter. The outer rim is bright yellow in colour. while centrally it is composed of loose haemorrhagic tissue within which is a small cavity . The ovarian foll icle transforms into a. corpus luteum after ovulation , during the course of which haemor rhage into the central cavity is invariable. The yellow rim represents the luteinised granulosa and theca cell layers, which at this stage secrete progesterone and , to a lesser extent, oestrogen in pre paration for implantation. If fertilisation does not occur, the corpus luteum involutes. Fig.S.37 Urethral valve. A flap of redundant epith elium is visible in the prostatic urethra (marked with a green probe); the bladder is trabecu lated and congested and there is marked bilateral hydroureter and hydronephrosis . This congenital mal formation is virtually confined to males and is one of the commonest causes of urinary obs truction in infancy. Age at presentation is dependent upon the degree of obstruction.
Fig.9.2 Corpora albicantia. This atrophic ovary from an elderly woman contains multiple small, yellowish-white nodules. Corpora albic anti a represent corpora lutea which have undergone physio logical involution, being replaced largely by hyalinised collagen : as such, they persist after the menopause when they become unduly conspicuous as a consequence of ovarian atrophy.
Fig.9.3 Cystic follicles. This bisected ovary shows several small, smooth-walled cystic spaces beneath the serosal surface . Such cysts represent germinal follicles which have undergone partiaf maturation, but then become atretic and cystic rather than rupturing . They are a common finding in perimenopausal women, in whom deteriorating ovarian function may be contributory. Occasionally they are associated with continued oestrogen secretion and thus may cause endometrial hyperplasia (see Fig.9 .30). Larger examples may be described as follicular cysts.
Fig.9.4 Tubo-ovarian abscess. The ovary and adherent fallopian tube (left) show extensive suppuration and haemorrhage. Such pyogenic oophoritis is most often associated with acute salpingitis . (see Fig.9.22) , but may occasionally result from haematogenous spread of infection from elsewhere. An important cause of infective oophoritis, although non-pyogenic, is mumps, which may impair fertility. Susprisingly, tuberculous salpingitis only rarely spreads to involve the ovary .
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9 Female Reproductive System
I
CLASSIFICATION OF OVARIAN TUMOURS
Serous cystadenoma/carcinoma Mucinous cystadenoma/carcinoma EPITHELIAL Endometrioid carcinoma Mesonephroid adenofibroma/carcinoma Fig.9.S Ovarian endometriosis (,chocolate' cyst). The bulk of this ovary is replaced by a haemorrhagic cystic cavity, filled with blood clot. Endometriosis (i.e. ectopic endometrial tissue outside the uterus) is commonest in the ovary, broad ligament and pouch of Douglas but may be seen almost anywhere. The aetiology is un· certain, but the ectopic endometrium undergoes normal cyclical changes, including menstrual bleeding which results in the formation of a 'chocolate' cyst. Interestingly, this condition is often cured by pregnancy,
Brenner tumour Granulosa cell tumour SEX-CORD STROMAL
Thecoma Hilar cell tumour (arrhenoblastoma)
I
Teratoma GERM CELL
Dysgerminoma Choriocarcinoma Fibroma/sarcoma
STROMAL MESENCHYME
Leiomyoma/sarcoma Lipoma/sarcoma
UNCERTAIN HISTOGENESIS Fig.9.S Serous cystadenoma. The ovary is replaced by a thin walled , fairly large unilocular cyst, over the surface of which the fallopian tube is stretched (bottom right). Serous cystadenomas are the commonest benign ovarian neoplasms and are derived from surface epithelium . They are typically smaller than their mucinous counterparts, are bilateral in up to 30% of cases and often show papillary excrescences on the internal surface.
73
Yolk sac tumour Secondary carcinoma primary
MISCELLANEOUS Lymphoma
secondary Fig.9,7 Classification of ovarian tumours.
Fig.9.S Mucinous cystadenoma. Replacing III" 1111111 V large, multiloculated , smooth cyst (top) ; the LJ"~IIII" 11111< Ii in the bottom right-hand corner for size compall','"1 I I, II, of this cyst 's lining shows multiple , smaliloc ul o~: lill, ill WI! Mucinous cystadenomas are common benin" Ii 11111 1111'·1, ' ovarian surface epithelium. They arise most ()1I"'i III II " decades , are bilateral in 10% of cases and ,llllY, 111 ,111 '
9 Female Reproductive System Fig.9.9 Serous cystadeno carcinoma. The ovary is replaced by a large unilocular tumour (top), the lining of which (middle) is composed of solid , papillary tumour showing haemor rhage and focal necrosis. Below, in a different example, tumour can be seen extending through the serosal surface. Serous cystadeno carcinoma is the commonest primary ovari an malignancy and is bilateral in up to 40% of cases. Women in the 6th and 7th decades are most often affected and 5-year-survival is only of the order of 25%. It is important to note that a g roup of serous and mucinous tumou rs of borderline malig nancy can be defined histo logically; these carry a much better prognosis.
IFICATION OF OVARIAN TUMOURS
Serous cystadenom a/carcinoma Mucinous cystadenoma/carcinoma
If IIAL Endometrioid carcinoma Mesonephroid adenofibroma/carcinoma Brenner tumour Granulosa cell tumour
onD MI\L
Thecoma Hilar cell tumour (arrhenoblastoma) Teratoma
LL
Dysgerminoma Choriocarcinoma Fibroma/sarcoma
~I\I
I ~CI IYM E
Leiomyoma/sarcoma Lipom a/sarcoma
tlAIN ( ,I NESIS
Yolk sac tumour Secondary carcinoma
I I I\NEOUS
primary Lymphoma secondary
'''cation of ovarian tumours.
Fig.9.a Mucinous cystadenoma. Replac ing the ovary is a ve ry large , multiloculated, smooth cyst (top); th e uteri ne fundus is visible in the bottom right-hand corner for size comparison. Below, a portion of this cyst 's lining shows multiple, smaillocuies filled with glairy fluid . Mucinous cystadenomas are common benign tumours, derived from ovarian surface epithelium. They arise most often in the 3rd and 4th decades, are bilateral in 10% of cases and may attain an enormous size. Rupl ure or leakage may give rise to pseudomyxoma peri tonei .
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9 Female Reproductive System
Fig.9.10 Mucinous cystadenocarcinoma. The ovary is replaced by a solid, haemorrhagic mass composed of multiple papillae and locules, containing viscid fluid. Mucinous cystadenocarcinoma is common, arising largely in the middle-aged or elderly and, like its benign counterpart, may attain a great size. It is derived from ovarian surface epithelium, as is its serous equivalent, but carries a better prognosis, with a 5-year-survival rate of up to 50% .
Fig.9.12 Brenner tumour. The ovary is replaced by a well circumscribed, irregular neoplasm, the cut surface of which is yellowish-white with mucoid and fibrous foci. Brenner tumours are derived from ovarian surface epithelium but show Wolffian differen tiation. They are comparatively uncommon but may arise at any age and are sometimes bilateral. In the vast majority of cases these tumours are benign.
Fig.9.11 Mesonephroid adenocarcinoma. Arising in the ovary is a large, predominantly solid, yellowish neoplasm which shows focal cystic change and necrosis. Mesonephroid (clear cell) adeno carcinoma is uncommon and is also derived from ovarian surface epithelium (despite its mistaken nomenclature). The clinical features are almost exactly the same as those of serous cystadenocarcinoma (see Fig. 9.9).
Fig.9.13 Granulosa cell tumour. The ovary is replaced by a large, well circumscribed, yellow tumour , within which are small foci of cystic change. Granulosa cell tumours, which are sex-cord stromal in origin, are relatively uncommon but arise most often in the peri menopasual years. They should be regarded as malignant (although often low-grade) and they typically secrete excessi ve oestrogens, leading to endometrial hyperplasia or carcinoma . Rare cases seen in young girls usually give rise to precocious pseudopuberty .
75
Fig .9.14 Thecoma. The cut surface of th is small, 'v' III, II I predominantly fibrous bul shows a typical area l)1V' .111 'WI ation , representing accumulated lipid Theco mw, " " ' ' ,. stromal tumours which most often arise periman!. '1"" ,... Iii are almost invariabty benign , they commonly S (~' ; II ' I" " , oestrogens which may result in the developmc llt I ,1, "1, Ii hyperplasia or carcinoma
Fig.9.15 Mature cystic teratoma. The ovary I" I' , I,. ·. , i I show replacement by a mullicystic tumour . wltlllil wl, " I , materia l and matted hair are evident. Mature CY'.II' I. 'I, II , benign ovarian dermoid ) IS a common germ (:,·11 11111 1/ \1" most often in the 2nd to 4th decades . It is r arll (,IILIlIV I ' II ' undergoing lorsion (see Fig .9.19). often bell HI II/ ·.III.1i II I ovarii is an uncommon variant of the same 1111111)'" " '" '1" dominantly of thyroid tissue. Malignanl ovamlll I· :1011. II " , uncommon (eI. testicular tera tomas)
9 Female Reproductive SYMt:!l1
renner tumour. The ovary is replaced by a well ,,,, I. Irreg ular neoplasm, the cut surface of which is Ifltll with mucoid and fibrous foci. Brenner tumours are " / Ivnrian surface epithelium but show Wolffian differen , !l U comparatively uncommon but may arise at any age " ,tJ' nes bilateral. In the vast majority of cases these I Hl nign.
Fig.9.14 Thecoma. The cut surface of this small ovarian lumour is predominantly fibrous but shows a Iypical area of ye llowish colour ation, representing accumulated lipid . Thecomas are sex-cord stromal tumours which most often arise perimenopausatly . While they are almost inva riably benign, they commonly secrete excessive oestrogens which may result in the development of endometrial hyperplasia or carcinoma .
nuloS8 cell tumour. The ovary is replaced by a large, rII ,ull , yellow tumour, within which are small foci of I IllInulosa cell tumours, which are sex-cord stromal in IIIVl!ly uncommon but arise most often in the peri ylll II n n 1ey should be regarded as malignant (although .10 ) I Hid they typically secrete excessive oestrogens, Ii IJIIOlrJ AI11yperptasia or carcinoma. Rare cases seen in I II Illy give rise to precocious pseudopuberty.
Fig.9.15 Mature cystic teratoma. The ovary has been bisected to show replacement by a mullicystic tumour, wilhin which sebaceous material and matted hair are evident. Mature cystic teraloma (or benign ovarian dermOid) is a common germ ceiliumour which arises mosl often in the 2nd to 4th decades . It IS particularly prone to undergoing lorslon (see Fig .9.19) , of len being pedunculaled . Struma ovarii is an uncommon variant of the same tumour , composed pre dominantly of thyroid tissue . Malignant ovarian teralomas are very uncommon (cf leslicular teratomas).
Fig.9.16 Dysgerminoma. Arising in this ovary is a large, uniform . well circumscribed. whitish tumour. similar in appearance to a potato . Dysgerminomas are comparatively uncommon germ cell tumours which show no identifiable differentiation. They are commonest between 10 and 30 years. are analogou s to the testicular seminoma (see Fig.1 0.3) and should be regarded as malignant. They are extremely radiosensitive and the prognosi s is excellent.
Fig.9.17 Fibroma. The ovary is replaced by a pale, lobulated tumour, the cut surface of which is fibrous and whorled. Ovarian fibromas are derived from stromal mesenChyme , usually arise in the 5th and 6th decades and are almost invariably benign . They may be associated with the development of ascites or pleural effusions (Meigs' syndrome), an entirely unexplained phenomenon .
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9 Female Reproductive System
Fig.9.22 Acute salpingitis. Serial sections tilrDlllIlIlI lI I tube show florid luminal suppuration and congcfllll ll' /'I, , salpingitis is most otten sexually transmitted : t~I U II ,, ;[.t I" . organisms responsible are chlamydia, mycopla',IIld I " " , The gonococcus is now thought to be only an il lit II 1111 " II ", predisposing factors include abortion, surgical "",1111111 11, the use of an intra-uterine contraceptive device .
Fig.9.18 Metastasis to the ovary. Both ovaries are diffusely replaced by pale , rather nodular tumour, in this case of breast origin Note also the follicular cyst (right). The ovary is not infrequently the site of metastasis, particutarly from primary carcinomas of th e endo metrium, gastro-intestinal tract and breast. Bilateral involvement is common. The term Krukenberg tumour is reserved only for those metastases , (most often of gastric origin) which histologically show a signet-ring, mucus-secreting pattern .
Fig.9.20 Tubal ectopic-pregnancy. This fallopian tube is dilated and the wall is thickened and haemorrhagic; within the lumen lies a tiny 9 week fetus. Ectopic implantation of the fertilised ovum is commonest in the fallopian tube, most otten in its ampullary portion. About 1 in 100 pregnancies are ectopic; possible causes include previous tubal inflammation or impaired tubal motility. Up to 60% of tubal ectopics rupture, usually by about the 12th week.
Fig.9_19 Torsion of ovarian cyst. This large ovarian cyst has twisted about the fallopian tube and broad ligament (top) resulting in tense, haemorrhagic engorgement. Ovarian torsion is not uncommon in association with an ovarian cyst or neoplasm, the size or weight of which results in twisting of the broad ligament or mesovarium. Peritonitis or gangrene may rapidly develop.
77
Fig.9.21 Paratubal cyst. This is a simple, smooth-walled , fluid filled cyst which was an inci dental finding in the broad ligament. Such cysts are insig nificant benign structures which may be derived from the ovarian hilum or from vestiges of the mesonephric (Wolffian), para mesonephric (Mullerian) or Gartner's ducts.
Fig.9.23 Chronic salpingitis. While the IUlno 11 1,11111' , I is focally patent (lett), the remainder is distortoli t 'y " t,l l l focally necrotic mas s. I n such cases, it is un(':Oll" II' "' " , infective organism but tubal damage such as till:, I' , h, I ' bilateral and may result in infertility. Macroscnl'il i llly , III salpingitiS may look very similar.
9 Female Reproductive System
Fig.9.22 Acute salpingitis. Serial sections through this fallopian tube show florid luminal suppuration and congestion . Acute salpingitis is most often sexually transmitted: the most frequent organisms responsible are chlamydia, mycoplasma or anaerobes . The gonococcus is now thought to be only an initiating factor. Other predisposing factors include abortion , surgical instrumentation and the use of an intra-uterine contraceptive device.
Fig.9.24 Pyosalpinx. Thi s fallopian tube is grossly dilated and distorted and the lumen contains yellowish , purulent debris . Note also the simple paratubal cyst (left). This appearance is another possible outcome of chronic salpingitis but may also be seen in very severe acule infection. Bilateral involvement is again common .
•Iblli ectopic-pregnancy. This fallopian tube is dilated I', 1I IIckened and haemorrhagic; within the lumen lies a 1111,. Ectopic implantation of the fertilised ovum is 111 1110 fallopian tube, most often in its ampullary portion. '11 11'l ognancies are ectopic; possible causes include II,,, lliammation or impaired tubal motility. Up to 60% of II" )Iure , usually by about the 12th week. Fig.9.21 Paratubal cyst. This is a simple, smooth-walled , fluid filled cyst which was an inci dental finding in the broad ligament. Such cysts are insig nificant benign structures which may be derived from the ovarian hilum or from vestig es of the mesonephric (Wolffian), para mesonephric (Mullerian) or Gartner's ducts.
Fig.9.23 Chronic salpingitis. While the lumen of this fallopian tube is focally patent (left), the remainder is distorted by a fibrotic and focally necrotic mass. In such cases, it is uncommon to isolate the infective organism but tubal damage such as this is frequently bilateral and may result in infertility. Macroscopically, tuberculous salpingitis may look very similar.
Fig.9.25 Hydrosalpinx. This fallopian tube is dilated and rather elongated and the lumen contains milky, clear fluid . Note the dense adhesions to paratubal structures. This appearance, which is seer:1 in the ampullary portion of the tube, is most often due to long-standing, low-grade infectio n but may also be associated with idiopathic pelvic inflammatory disease.
78
9 Female Reproductive System
Fig.9.28 Localised adenomyosis, Thi s sagittal section through a uterus shows a well circumscribed, whorled mass in the anterior wall, within which there are haemorrhagic and yellowish areas. Adenomyosis is defined as the presence of endometrium deep in the myometrium and is commonest in parous peri menopausal women. It results from downward extension of the basal endometrium and either involves the uterus diffusely or is localised, as here , when it may be known as an 'adenomyoma'. The aeti ology is possibly related to excessive oeslrogenic stimulation .
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Fig.9.26 Bicornuate uterus. This specimen shows two apparently normal uterine cavities which converge on a single cervical ca nal. This is a congeni tal malformation, representing partial failure 01 Ius ion 01 the Mullerian ducts . Up to 1 in 500 women have developmental anomalies of varying severity in the genitaltraCl, which may give rise to menstrual or obstetric problems.
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Fig.9.27 Endometrial polyp and Nabothian cysts. This coronal section of the uterus shows a polypoid lesion arising Irom the endometrium: in addition there are several smoo th cystic cavities in the endocervix. Endometrial polyps are common, most often peri menopausal, lesions which may be multiple. Whether they represent true benign neo plasms or a localised reaction to oestrogen hypersensitivity is uncertain. Nabothian cysts represent cystic dilation of endocervical glands and are of no known signilicance .
79
Fig.9.29 Uterine leiomyomata. The uterus on the lett shows several well circumscribed, whorled fibrous neoplasms w',thin the myometrium; that on the right shows a single, pedunculated sub mucosal tumour of a similar nature which is distorting the endometrial cavity. Uterine leiomyomata ('fibroids') are very common benign tumours of smooth muscle which develop during the reproductive yea rs, probably as a result of oestrogen sensitivity. Degenerative changes, including calcification or infarction , are common.
F,i g.9.31 Endollllll, l carcinoma. AIII,I' II II, endometrilill "" II" I , uterus is a 1;11'1' ' . 1" 11 locally neCl ol" '" ' I 'I ' Endometri;II I..1I 1"",,, uncommon Hilt 1_" , II often after 1111 ,,,,,,, 11 '1 ' Proven 3 !;S()( .10I11i ",, I null iparity , 1I1t. " llId all of whir.l< ;111'11""" 1 cool actors 01,' " , " '110 1 oestrogen ~~ llIlI lI loI lil li year -survlvill 1.11"" " of 70%, dul" ,,,01,,, II " logica l (Jraclc ' d /lil "t ,
9 Female Reproductive System
Fig.9.28 Localised adenomyosis. This sagittal section through a ulerus shows a well circumscribed, whorled mass in the anterior wall, within which there are haemorrhagic and yellowish areas. Adenomyosis is defined as the presence of endometrium deep in the myometrium and is commonest in parous peri. menopausal women . II re sults from downward extension of the basal endometrium and either invo lves the ulerus diffusely or is localised, as here, when il may be known as an 'ad enomyoma'. The aetiology is possibly related to excessive oestrogenic stimulation .
lerlne leiomyomata. The uterus on the left shows several I .li l II ,el. whorled fibrous neoplasms within the II 111.11 1.) 11 Ihe right shows a single , pedunculated sub '" " " (,I ;) similar nature which is distorting the endometrial 'II' I', il(ilTlyomata ('fibroids') are very common benign It il ,,,Iii muscle which develop during the reproductive ., Il y , I~'
Fig.9.30 Endo metrial hyper plasia. Within the body of the uterus. the endometrial lining shows irregular, almost polypoid thickening. Endometrial hyper plasia , which is associated with cystic dilatation of endometrial glands, is due to unopposed oestrogen stimula tion as may occur in anovulatory menstrual cyc les or in association with ovarian sex-cord stromal tumours. Histologically atypical var iants of this condition may be associaled with the development of endometrial adeno carcinoma. Fig.9.31 Endometrial adeno carcinoma. Arising from the endometrium In the body of the uterus is a large, polypoid . focall y necrotic neoplasm . Endometrial carcinoma is nol uncommon and occurs most often after the menopause. Proven associations include nulliparity. infertility and obesity, all of which are thought to be co-factors of ex ces sive oestrogen stimulation. The 5 year-survival rate is of the order of 70%, dependent upon histo logical grade and staging.
Fig.9.32 Malignant mixed Mullerian tumour. Ari sing in the uterine fundu s is a large, polypoid, haemorrhagic mass. Extensive myometrial invasion is apparent. Malignant mixed Mullerian tumours are derived from Mullerian mesenchyme and thus contain both epithelial and connective tissue elements. They arise most often in the elderly and car ry a poor prognosis . Mixed Mullerian tumours may some time s have only one malignant com ponent , which is usually mesenchymal , (adenosarcoma) or be entirely benign (adenofibroma) Fig.9.33 Uterine leiomyosarcoma. In the myometrium of the fundus and body is an irregular, pale neoplasm showing focal haemorrhage and necrosis . Serosal invasion is apparent (top) . Leiomyosarcoma of the uterus is comparatively rare, usually occurs in the 5th and 6th decades and may be associated with nulliparity . These tumours arise de novo and not from leiomyomas : the 5-year-survival is about 30%.
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9 Female Reproductive System
Fig.9.34 Hydatidiform mole. This specimen consists of a mass of grape-like hydropic villi which has been removed from the uterine cavity. Hydatidiform mole arises in about 1 in 1,500 U.K. pregnancies but is much commoner in the Far East. Such moles are derived from abnormal trophoblastic proliferation and usually affect females at the extremes of reproductive life. Up to 3% go on to develop chorio carcinoma and therefore careful follow-up by serum r3-HCG estimations is mandatory.
Fig.9.35 Choriocarcinoma. This uterus has been opened to show diffuse replacement of the myometrium by an extensive haemor rhagic tumour. Choriocarcinoma is a malignant tumour of tropho blast, the incidence of which parallels that of hydatidiform mole, since 50% of cases are preceded by a mole. It may also arise following spontaneous abortion or a normal pregnancy. Despite a tendency to extensive vascular invasion, up to 90% of affected patients are cured by chemotherapy in conjunction with careful follow-up using r3-HCG estimations.
81
Fig.9.36 Cervical 'erosion'. Around the external os, the cervical epithelium appears reddish . This appearance is, in fact, physio logical and is not due to epithelial erosion at all. Rather, it is due to eversion of endocervical mucosa, which occurs with elongation of the endocervical canal during adolescence, and which then undergoes squamous metaplasia.
Fig.9.37 Endocervical polp. A close-up view of the endocervical canal and vaginal vault shows a small, rather mucoid polyp in the endocervical canal, surrounded by operative haemorrhage . Endo cervical polyps are extremely common, occurring most often in the 4th and 5th decades, and represent focal hyperplasia of the endo cervical epithelium Superimposed inflammation is common but malignant change is excessively rare.
Fig.9.38 Cervical squamous carcinoma. Ari si! I! I 1,, 11 11 Ii ectocervi x is an irregular, fungating, pale neopIBn", ( "" . the cervix is common and Increasing in incidenCll , wll,l!! Ii most frequently in the 5th and 6th decades, yountJi If w',, ,, , infrequently affected. It is thought to arise in prO-OXIIII' \f11" intra-epithelial neoplasia (dysplasia) over a peri'" I " I 1111, Aetiologically, Herpes simplex type II and human 111 11"II, ii' types 16 and 18 are thought to be important, witl'l ,)fllly ,II coitus being the most critical co-factor. Overall !J yonl '"'' about 50%, dependent upon the degree of inva~ i,,, I
9 Female Reproductive System Fig.9.40 Vulval Bowen's disease. This simple vulvectomy
Fi~.9.38 Cervical squamous carcinoma. Arising from the eDtocervi x is an irregular, fungating, pale neoplasm. Carcinoma of th~ cervix is common and Increasing in incidence; while it occurs m()st frequently in the 5th and 6th decades, younger women are not infrequently affected. It is thought to arise in pre-existent areas of intra-epithelial neoplasia (dysplasia) over a period of 10 to 20 years. A~tiologically, Herpes simplex type II and human papillomavirus tY10es 16 and 18 are thought to be important, with early age at first COitus being the most critical co-factor. OveraIl5-year-survival is about 50%, dependent upon the degree of invasion.
Fig.9.39 Vaginal squamous carcinoma. Arising in the posterior vaginal wall is a raised, irregular neoplasm. Squamous carcinoma of the vagina is a rare tumour of the elderly, which is probably aetio logically similar to cervical carcinoma. Vaginal adeno carcinoma is also extremely uncommon but is typically a tumour of young girls, whose mothers were treated with di· ethylstilboestrol during pregnancy.
specimen shows a raised, rather nodular, reddish area around the inferior margins of the introitus. Vulval Bowen's disease (intra epithelial neoplasia) represents carcinoma-in-situ and is not uncommon, particularly with increasing age. Aetiologically, the viruses implicated in cervical carcinoma are thought to be important (see Fig.9 .38) . Progression to invasive carcinoma is not uncommon but occurs most often in the elderly or immunosuppressed.
Fig.9.41 Vulval squamous carcinoma. The labia majora of this radical vulvectomy specimen are diffusely infiltrated by a raised, nodular and focally ulce rated neoplasm . Vulval carcinoma occurs most often in the 7th and 8th decades, usually in cases with pre existent intra epithelial neoplasia or chronic inflam mation.OveraIl5 year-survival is about 70%. A rare, very well differen tiated warty variant, . known as verrucous carcinoma, only very rarely metastasises and has a better prognosis.
82
10 Male Reproductive System Fig.10.1 Testicular atrophy. A co ronal section through Ihi s tes li s shows Ihat it is much smaller than normal. part ic ularly whe n compared to the sp ermatic cord (abov e). We ll re cog nised ca uses of testicular at roph y include matdescenl. oestrogen the rapy , alcoholism, irradiation, Klinelelter's syn drome and hormonal abnormalill es olthe hypothalami c-pi tu itary-adrenal axis. Such tes tes typ ica ll y show markedly diminished or absent sp ermatogenesis.
CLASSIFICATION OF TESTICULAR TUMOURS classical
38%
Seminoma
1i11"lh , wtll il I :1(111 11 11
spermatocytic
2%
, ~ III II \
!t " 41111 11111 1 \ 1'
differentiated
1%
1 11' 1111 11
111 ' 111 II IH
GERM CELL
intermediate
20%
Teratoma undifferentiated
III
,11 ,11 1"
10%
II "II'" . III ' d ~
Ii
, I~ ".1
I
!l II IV
'1111 ', 1,
trophoblastic
1%
/1 '111011, , IY" I I' 1"1 ' I :
Combined seminoma! teratoma
14%
~.
II I Iii
!' . ' "
11
'11", '.11 ' ,1/ ' VI'
NON-GERM CELL
Yolk sac tumour
1%
Leydig cell tumour
2%
Sertoli cell tumour
1.5%
primary Lymphoma
7%
secondary OTHER Fig.10.2 Testicular torsion. The testis, epididymis and distal spermatic co rd show dark haemo rrhagi c infarc tion. This condi tion is, in fact, due to torsion of the spermati c cord , in whi ch ve nou s obstruc tion occurs lirst, resulting in intense distal congestion. It is . co mmonest in the 2nd decade and is usua lly due to an abnormally long or malorientated spe rmat ic cord or mesorchium . Early diagnOsi s may allow preservation 01 testicu lar lunc tion and co ntralateral orchidopexy sh ould always be perfo rmed.
83
Leukaemic infiltration
<1 %
Metastases
< 1%
Fig.10.3 Classification of testicular tumours (mod ifi ed infor· mation from the U.K . Testicu lar Tumour Panel ) Thes e ligu re s represent percentag es of the total number of testicular tumours.
I! Ii F.I '
Fig.10.5 0111.-"" , teratoma.'W,lI iII ' II we ll circlllII'" III" , is multicystl' .11" I, yellowisl-I kl ' /.,111 " " Differenti;ltl ', 1," '1,1 uncommOIl, ,II i I II about 2% "I I, " ,,", teratomas, ;11 II I" I age from 1," 11 1' " are comp"',' 'I I ' j ' ,I tissue Iron I, "' V' II germinal Illyl II' I I II apparentl y I II '1 11i I' 10% mel a" t. I' ,"II those OCC'"I11"I "
10 Male Reproductive System
~
.::iIFICATION OF TESTICULAR TUMOURS
classical
38%
Seminoma
H~ E LL
spermatocytic
2%
differentiated
1%
intermediate
20%
undifferentiated
10%
Teratoma
trophoblastic
1%
Combined seminoma! teratoma
t ri M
14%
Yolk sac tumour
1%
Leydig cell tumour
2%
Sertoli cell tumour
1.5%
primary Lymphoma
7% secondary
Leukaemic infiltration
< 1%
Metastases
< 1%
.,lftcation of testicular tumours (modified Infor I,,' U 1< , Testicular Tumour Panel). These figu re s 'IfItiJ £leS of the total number of lesticular tumours.
Fig.10.4 Seminoma. Much of this testis is re placed by a well circumscribed, lobulated pinkish white tumour. Seminomas are the commonest p rim ary testicular tumour and are derived from germinal cells. They occur most often in the 4th decade and are the most frequent tumours to arise in an un descended te stis. They are indistin guishable from the female ovarian dysgerminoma (s ee Fig.9 .16). Seminomas are extreme ly radio sensitive an d 5-year survival is now at least 90%. Fig.10.5 Differentiated teratoma.With in this testis is a we ll circ umscribed mass wh ich is multicystic and contains yellowish keratinous debris . Differentiate d teratomas are uncommon , accounting for only about 2 % of testicular teratomas, an d may arise at an y age from b irth to 30 years . They are com posed solely of mature tissue from any of the three germinal layers but, despite their apparently ben ign na tu re . up to 10% metastasise, pa rticularly those occurring in young adults.
Fig.10.6 Malignant teratoma inter mediate (MTI) Th e lower pole of the teslls is replaced by a multinodular. rather necroti c tumour , above which is an extensive zone of infarction due to torsion . MTI, which retains some differenti ated foci (in cont rast to MTU) is typically a tumour of young adu lt males. It tends to both haematogenous an d lymphatiC sp read and the 5-year survival is about 55% (beller th an MTU or MTT). Note that any teslicular tumour may predi spose to, and occasionally present wi th. torSion
Fig.10.7 Malignant teratoma trophoblastic (MTT). Th is testis is complete ly replaced by a multinodu lar, dark, haemorrhag ic mass showing paler foci of necrosis. MTT (or choriocarci noma) is the rarest subt ype of teratoma and is typified by the presence of syncytio trophoblast and cytotrophobl ast. Characteristicall y this tumour is very haemorrhagic. The prognosis was previously abysm al, but the use of both modern multimodal th erapy and p-human chorionic gonadotrophin as a serum marker has led to greatly improved survival.
84
10 Male Reproductive System
85
Fig.10.8 Chronic epididymitis. The epididymis and distat spermatic cord are markedly thickened and fibrotic and in places , obstructed ducts are visibly dilated . There is secondary testicular atrophy. Epididymitis is usually associated with lower urinary tract infection or urethritis and is commonest in adult hood. The condition is most often uni lateral and may spread locally to the testis or tunica vaginalis. Recurrent infection and chronicity are not uncommon.
Fig.1 0.10 Tuber culous epi didymitis. The epi didymis is totally replaced by an irregular cavity containing copious caseous material. The testis is com pressed but completely spared. Tuberculous epi didymitis is most often seen in young adults, usually in association with TB of the urinary tract: it has become very uncommon in the indigenous Western population Bilateral infection is frequent and while the sper matic cord may be affected, the testis is onty rarely involved .
Fig.10.9
Epididymal abscess. The epididymis appears rather thickened and scarred but in addition, there is a small central abscess cavity with adjacent conges tion . The testis shows partial atrophy. This is an example of acute on-chronic infection which , in very severe cases, may be complicated by abscess formation. Gonococci are often isolated from such a lesion.
Fig.10.11 Prostatic adenocarcinoma. A transverse section through the whole prostate gland, near the bladder neck, shows diffuse replacement by pale, irregular and focally necrotic tumour. Residual nodular areas of benign tissue are also present. Prostatic car cinoma is very common from the 6th decade onwards but often pursues an unaggressive course. The aetiology may be hormonal. The outer prostatic glands are the usual site of origin, particularly in the posterior tobe. A raised serum level of tartrate-labile acid phosphatase is a useful diag nostic marker and metastasis to bone is a characteristic feature of this tumour.
Fig.10.12 Benign prostatic hypertrophy. AlII 'Vi ' 11" bladder of an elderly male are shown. Til e PI UI,I. II I ' I', I, with a particularly prominent median lobe ul1~: tll" '" " 111 1 neck. The bladder wall is thickened and tr;.\l 1\)(.1II. ", ,, ' I transverse section through a retropubic rred l il l II tlllli V shows marked enlargement, the prostatic tll a,II" I 11111 II I ' muttiple yellowish-white nodules. Benign pn ,' ,1. 1111 II VI " (nodular or myoadenomatous hyperplasia) 1', IIIC" ', ,',11" with advancing age, being almost univers(1i I 'V II I" ' 11 1, , due to an, as yet unclear, imbalance b etwc\ 'I I I, " Ill " ,1, " oestrogen. The inner group of prostatic ql;lI lr l' , ,,'" IVI,I, leading to urinary obstruction . It does not p" "II' .1 P" I, "
10 Male Reproductive System Fig.1 0.1 0 Tuber culousepi didymitis. The epi didymis is totally replaced by an irregular cavity containing copious caseous material. The testis is com pressed but completely spared. Tuberculous epi didymitis is most often seen in young adults, usually in association with TB of the urinary tract it ... has become very uncommon in the indigenous Western population. Bilateral infection is frequent and while the sper matic cord may be affected, the testis is only rarely involved.
Fig.10.11 Prostatic adenocarcinoma. A transverse section through the whole prostate gland, near the bladder neck, shows diffuse replacement by pale, irregular and focally necrotic tumour. Residual nodular areas of benign tissue are also present. Prostatic car cinoma is very common from the 6th decade onwards but often pursues an unaggressive course. The aetiology may be hormonal. The outer prostatic glands are the usual site of origin, particularly in the posterior lobe. A raised serum level of tartrate-labile acid phosphatase is a useful diag nostic marker and metastasis to bone is a characteristic feature of this tumour.
Fig,10.13 Penile squamous carcinoma. Arising at the base of the glans penis (top) is a pale , ulcerating neoplasm, which is better seen in cross section (bottom) . Squamous car cinoma ot Ihe penis is relatively un common in the Western World, but tends 10 arise in elderly uncir cumcised men . In some cases there may be pre-existent Bowen's disease. The majority of these tumours develop from the glans peniS or the prepuce While this tumour lends to exophytic or locally invasive growth , metastasis is usually a late phenomenon. How ever, presentation is often delayed by the patient's shyness or mistaken belief that he has a venereal disease.
Fig.10.12 Benign prostatic hypertrophy. Above , the prostate and bladder of an elderly male are shown . The prostate is hypertrophied with a particularly prominent median lobe obstructing the bladder neck. The bladder wall is thickened and trabeculated. Below, a transverse section through a retropubic prostatectomy specimen shows marked enlargement, the prostatic tissue being composed of multiple yellowish-white nodules. Benign prostatic hypertrophy (nodular or myoadenomatous hyperplasia) is increasingly common with advancing age, being almosl universal by the 9th decade. It is due to an, as yet unclear, imbalance between testosterone and oestrogen. The inner group of prostatic glands are typically affected, leading to urinary obstruction . It does not predispose to carcinoma.
86
11 Nervous System
[
CAUSES OF
~Y~~~C~P~A~U~
--- - - - - ]
r
I
Arnold-Chiari malformation Bifid aqueduct
CONGENITAL
Atresia of 4th ventricular foramina (Dandy-Walker syndrome)
Intra-uterine infection Fig.11.1 Hydrocephalus. This coronal seclion of brain shows gross dilation of the lateral ventricles , due to obstruction of the cerebral aqueduct by a glioma . Hydrocephalus may be classified into 4 types Non·communicatmg (internal) due to obstruction of the aqueduct or foramina of the fourth ventricle; Communicating due to obstruction at the subarachnoid cisterns; External due to impaired reabsorption of CSF; and Compensatory, associated with cerebral atrophy.
toxoplasmosis syphilis
Posterior fossa tumours ACQUIRED
Post·meningitic Compensatory
FLOW OF CEREBROSPINAL FLUID pineal gland
palhol cereb rospinal fluid
[J
arac',nOld mater
D
ependyma
choroid plexus
lateral ventricle
Interventricular foramen
Fig.11.5 Adult bacterial meningitis. The SUP ' , II
Fig.11.3 Causes of hydrocephalus.
-t-...2 ~f :"\
~) ~. "
--
anterior horn third ventricle
Inferior horn pituitary gland
{'ft cerebello medullary cistern median aperture
JJ
lateral aperture pons
::-=-s ......
pontine cistern
ifF
medulla
Fig.11.2 Simplified representation of flow of cerebrospinal fluid, seen In the left hemisphere (medial vi ew) .
87
Fig.11.4 Syringomyelia. This segment of cervical spinal cord contains a dilated, cystic cavity (syrinx), which has compressed the central canal anteriorly to a barely discernible slit. Possible associa· tions include four1h ventricle out flow obstruction, resu lting in hydromyelia, and neurofibroma tosis (see Fig . 11.28) Typica l clinical features include impaired pain and temperature sensations and the 'claw-hand' deformity, due to nerve tract compression
Fig.11.6 Neonatal bacterial meningitis. 1111' '.1 II I,II " ' neonate's brain shows congestion and SUPI "II"III \II , " 'I I the inferior aspect of the right temporallot,r. NOI,,,,,I, ,I , seen most often after a prolonged or traurn;III' . ' 1" ItV' ''''' common in premalure infants. The culpabllo) nIC Ii III" ,II I I' 1 derived Irom the maternal genital tract at 1' 1111>, 1110'.11 11 Streptococcus pyogenes or a coliform, pallll:tll.llly f •
11 Nervous System
Ij
Fig.11.S Adult bacterial meningitis. The superior surface of the brain is intensely congested and covered in a purulent exudate , particularly over the frontal lobes (left) . Suppurative meningitis may complicate endocarditis, middle ear, sinus or pulmonary infections or trauma. Direct spread of organisms may also occur from the nasopharynx . Important pathogenic organisms include Neissena meningllidiS (in young chitdren and young adulls) , Haemophilus influenzae in young children and Streptococcus pneumoniae in the very young or old . Almost any organism , including fungi . may be responsible in the immunocompromised patient
Fig.11 .6 Neonatal bacterial meningitis. The surface of this neonate's brain shows conges tion and supp uration, especially over the inferior aspect of the right temporal lobe. Neonatal meningitis is seen most often after a prolonged or traumatic delivery and is more common in premature infants. The cu lpable organism is always derived from the maternal genital tract at birth, most often being Streptococcus pyogenes or a coliform, parti c ularly E. coli.
Fig.11.7 Tuberculous meningitis. Over the parietal lobe there is a dense inflammatory exudate associated with numerous adjacent 'tubercles' . Tuberculous meningitis is usuall y seen as a complication of primary Infection in young individuals and is most often due to miliary spread. It may also result from rupture of a localised intra cerebral tuberculous (Rich) focus into the subarachnoid space The base of the brain and upper cerebe llum are most often affected . Fig .11.8 Cerebral abscess. Within this left cerebral hemi· sphere is an irregular abscess cavity which is parlly walled off . There is surrounding con gestion Predi sposing causes are much the same as those for suppura· ..... ~~ tive meningitis (see Fig.11.5) in cluding haematogenous spread of any sys tem ic Infec tion. The latter typically leads to abscesses localised in the distribution of the middle cerebral artery.
88
11 Nervous System
Fig.11.9 General paresis of the insane. This coronal section of brain shows marked cortical atrophy , flattening of the gyral pattern and compensatory hydrocephalus . General paresis is a late (quaternary) manifestatioll of syphilis . Other macroscopical features include leptomeningeal thickening and granular ependymitis. The resultant neuronal loss may be associated with dementia, epilepsy, motor dysfunction and the Argyll Robertson pupil. Cord involvement in quaternary syphilis gives rise to tabes dorsalis .
Fig.11.10 Raised intracranial pressure. This coronal section of brain shows marked compression and asymmetry of the left lateral ventricle and, just above, herniation of the cingulate gyrus. Raised intracranial pressure is most often seen in association with intra- or extracerebral haemorrhage, a tumour or extensive infarction . The cardinal macroscopic features, other than those shown here, are tentorial or tonsillar herniation (cerebellar coning) and uncal grooving ,
89
Fig.11.11 Cerebral fat embolism. This section of brain shows numerous small petechial haemorrhages, most notably in the white matter. Fat embolism most commonly results from damage to a major bone , particularly a fracture, in which medullary fat enters the venous system. This may pass unnoticed or may result in impaired cerebral, pulmonary and renal function, Other causes of such haemorrhages include malaria, leukaemia and thrombocytopenic purpura.
Fig.11.12 Acute subdural haemorrhage. The specimen consists of dura (right), blood clot and the left cerebral hemisphere. An extensive depression is visible in the left temporoparietal region. Subdural haemorrhage is usually traumatic in origin and results from tearing of thin-walled veins as they enter the dural sinuses. Acute lesions may be rapidly fatal if not surgically evacuated, while undetected haemor rhage may result in gradual, chronic cerebral damage.
Fig.11.13 Subarachnoid haemorrhage. Ruplill" 1.1 .1I " aneurysm of the left posterior cerebral artery 1I1I:; 1/1 '.1 III. I' I subarachnoid haemorrhage around the base 0111 11 ' 11111 111 tensive rupture of berry aneurysms (see Fig .1! ! ~l) I'. II " source of subarachnoid bleeding but other imiJl >Iii" II , •I' trauma and extension of an intracerebral haem(1I II 1,," 11 prognosis is generally poor .
Fig.11.14 Cerebral artery aneurysms. On Il u' II ,II, 11 11 have been separated to display a berry aneuIY:lIl l (11111 '1 at the junction of the left anterior cerebral and :1111 11111 II' , cating arteries . On the right, an atheromatous ~.j\\ ! qlill (see Chapter 1) is seen in the left posterior C()11l1 II III ," II Berry aneurysms usually result from degenornll vl' 1Ii,1I1 of a congenital defect in the arterial wall. They frill Y I." II are sometimes associated with polycystic r91l; 11dlllllll'" subarachnoid haemorrhage, they may alsOhi', Gi ll ""III cerebral haemorrhage or infa,rction.
I I Nervous System
. ....c
"" Fig.11.17 Recent cerebral infarct. There is an extensive area of haemorrhagic infarction with a hyperaemic border in the parietal region. Note also the adjacent marked cerebral oedema. Cerebral infarction is the commonest cause of a 'stroke' (CVA) and is most often due to thrombosis in an atheromatous vessel. It may also be embolic in origin (e .g . from the lett atrium in atrial fibrillation) or associated with hypercoagulability or the contraceptive pill. Fig.11.18 Old cerebral infarct. This coronal section of brain shows a massive previous right -sided inlarct which has resulted in loss 01 cortical tissue, cysl ic degeneration and compensatory hydrocephalus. Multiple (usually small) inlarcts over a variable period 01 time may lead to numerous loci 01 cerebral softening (status spongiosus) and Ihe clinical syndrome'ol mull, inlarct dementia.
91
Fig.11.19 Multiple sclerosis. tn the periventricular white matter and adjacent internal capsule (left) there are three well -defined grey plaques (arrowed) indicati ve of foci of demyelination and gliosis . Multiple sclerosis typically affects young adults, particularly females and is a chronic. relapsing and debilitating disease . Aetiologically, a slow viral infection is thought (but nOI proven) to be responsible , perhaps in combination with genetic factors. Fig.11.20 Alzheimer's disease. The meninges have been stripped from the left side of this brain to show marked cerebral atrophy , manifest by sulcal widening and diminution of the gyri. Alzheimer's disease is a chronic form of pre-senile dementia, is commonest in the 5th and 6th decades and may also be seen in Down's syndrome. The aetiology is entirely unknown.
Fig.11.21 Meningioma. A circumscribed nOIIlII;1I tlill II III I from the meninges (left) has been 'shelled out' nll ll(1 " ' 11 1' leaving a deep spherical depression . MeningiC') lnlUl , d. ,,,11' arachnoid villi, most often arise in relation to 111 0 lI1.'i, 1I \I",,' sinuses. They are slow-growing, almost invanailly 1"!I l1 lJ,1 but may occasionally invade the adjacent skllil . 111I 'V"1 , I I dominantly in the 5th and 6th decades and SYflli>I ()1I 1~.. II ' upon the site of the tumour.
Fig.11.22 Glioma. This coronal section of b, Llil •. .1 II I....,. " neoplasm, with foci of haemorrhage and necro::i·. III It lll I, sphere . There is adjacent oedema and distortioll ()III ~I .I' system. Gliomas may be divided, in order of froql I, "ILV II blastoma multiforme (see Fig 11.23), astrocyl \lIl .fI, 11111t1 ., I glioma, ependymoma and choroid plexus pal)illol lIit M I not uncommon . Gliomas do not give rise to Sy ~tl1JJ1I1 Iq, ·1 may 'seed' throughout the CNS and cause de:lll. hy Ih, II effects.
11 Nervous System
matter and grey gliosis larly females Irolog ically, a ponsible,
Fig, 11,21 Meningioma, A circumscribed nodular tumour arising from the meninges (left) has been 'shelled out' of the left parietal lobe, leavi ng a deep spherical depression. Meningiomas, derived from the arachnoid villi, most often arise in relation to the major venous sinuses. They are slow-growing, almosl invariably benign, tumours but may occasionally invade the adjacent skull. They occur pre dominantly in the 5th and 6th decades and symptoms, if any, depend upon the site of the tumour.
Fig.11.23 Glioblastoma multiforme. These coronal sections of brain show a massive haemorrhagic tumour arising in Ihe basal ganglia and dislorting the lateral ventricles . Glio blastoma multiforme is an un differentiated glial tumour, most often of aslrocytic derivatio n, and occurs predominantly in the 4th and 51h decades. It is the commonest variant of glioma, arises mosl often in the frontal lobes, septum pellucidum and basal ganglia and carries a very poor prognosis .
.
"Y
/~; ~ ~. ' y.
.
.
,
Fig.11.22 Glioma. This coronal section of brain shows an ill-defined neoplasm. with foci of haemorrhage and necrosis in the left hemi sphere. There is adjacent oedema and distortion of the ventricular system. Gliomas may be divided, in order of frequency, into glio blastoma multiforme (see Fig 11.23), astrocytoma, oligodendro glioma, ependymoma and choroid plexus papilloma. Mixed types are not uncommon. Gliomas do not give rise to systemic metastases but may 'seed' Ihroughout the eNS and cause death by their local effects.
Fig.11.24 Ependymoma. Arising in the 4th ventricle and compress ing the cerebellum posteriorly is a large, multUobulated, while tumour. Ependymomas are one of the least frequent forms of glioma but are the commonest to arise in the spinal cord. They are derived from the ependymal cells that line the ventricular system and cord canal. While typically slow-growing , their location often renders Ihem in operable and the prognosis is poor.
92
II
11 Nervous System
Fig.11 .25 Acoustic neuroma. Situated on the left cerebello· pontine angle is a well circumscribed neoplasm arising from the eighth cranial nerve . This is a benign Schwann cell tumour which is sometimes bilateral and may be associated with neurofibromatosis . Clinical features include tinnitus, vertigo and nerve deafness . Compli cations include compression of other cranial nerves or of Ihe brainstem.
" tll'W'dl l t
I n,
I tl d
- I II
I
11 , 1, 11 11'
(I ,,,lr'W I
1111 ' 1 I" II" 1111 11 lit I I i
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I
~ ;111 11 Ii 11 II
III '.rl I IV II "
111 111" , , 11 I 1111 11 11111 1, 11
I .ril,,' Wi ,
Fig.11.27 Benign schwannoma (neurilemmoma). This is a well circumscribed, encapSUlated, small tumour which has a yellowish cut surface and shows small foci of haemorrhage. Benign schwannoma is a common tumour of peripheral nerves and arises from the nerve sheath. It is usually solitary, arises especially in the 3rd to 5th decades and is most often asymptomatic Occasionally, multiple lesions may be seen in neurofibromatosis but in the latter condition multiple neurofibromas are far more common.
" IIiI1WII I I I ( ,lIllh \ ~ Ii
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Fig.11.26 Cerebral metastasis. This coronal section of brain shows a solitary, large deposit of focally necrotic and haemorrhagic meta static tumour , which is situated in the region of the left basal ganglia and is compressing the lateral ventricle . Cerebral metastases, which are usually multiple, are most often situated at the junction of the grey and white matter and are typically well circumscribed (cf. glial tumours). The most frequent primary sites are bronchus, breast, kidney and cutaneous malignant melanoma .
93
Fig.11.28 Plexiform neurofibroma. This is a major pelvic nerve trunk from a patient with neurofibromatosis and shows gross thicken ing and expansion by a diffuse tumour. Neurofibromas are benign tumours of nerve sheath origin . They are often seen in von Reckling hausen's neurofibromatosis, an inherited condition which is charac terised by cafe au lait spots, multiple peripheral nerve tumours and is associated with CNS tumours, phaeochromocytoma and an increased risk of developing neurofibrosarcoma .
12 Osteoarticular System Fig.12.1 Bone fracture. Above, the proximal femur shows an obvious recent subcapital fracture. Part of a rib (below). adjacent to the costochondral junction, shows a transverse fracture, on either side of which is exuberant callus formation. Simple fractures heal by the produc tion of periosteal and then medullary callus. which is followed by new cartilage and bone formation with sub sequent re modelling. The majority of fractures are traumatic in origin but a minority occur through a pre exislent pathological lesion (e.g. a metastasis) Complications include deep ver,ous thrombosis, fat embolism and damage to adjacent tissues (e.g. muscle, tendon, vessels), the latter sometimes leading to Volkmann's cont ractu re.
Fig.12.2 Malunion. This macerated segment of a long bone shows clear evidence of a previous fracture but. malunion has occurred with de formity resulting from overlap of the bony ends. Malunion follows failure to reduce a displaced fracture. This appearance may also be seen, in a less extreme form, after fracture through an epiphyseal plate (prior to completion of ossification) which results in an abnormal growth pattern.
Fig.12.3 Non-union with false joint. Following a fracture of this humerus the bony ends have not been apposed. This has resulted in fibrous union, succeeded by cartilaginous metaplasia and the formation of a pseudarthrosis. Causes of non-union include delayed union (most often due to ischaemia, excessive mobility, local infection or malnutrition), the presence of extraneous tissue between the bone ends or failed treatment of an extensive or widely displaced fracture.
94
12 Osteoarticular System Fig.12.4 Chronic osteomyelitis. The shaft of this macerated femur shows extensive necrosis (the sequestrum) and is surrounded by a dense outer shell of periosteal new bone (the in vOlucrum) . Chronic osteomyelitis most often follows an untreated acute episode this latter is commonest in children and is usually the result of a bacter aemia (often staphylococcal, streptococcal or pneumo coccal). Salmonellae may be responsible in patients with sickle cell anaemia. Infect ion commences in the highly vascular metaphysis. most often of a long bone, and is followed by subperiosteal and intra medullary spread. Fig.12.5 Spinal tuberculosis (Pott's disease). This portion of the thoracic spine shows caseous necrosis of two adjacent vertebral bodies with destruc tion of the inter vertebral disc . Tuberculous osteomyelitis, although commonest in long bones, shows a predilec tion for the spine. It is most often a result of spread from primary pulmonary infection and may be complicated by vertebral collapse, a paravertebral 'cold' abscess or cord compression .
...
Fig.12.6 Syphilitic periostitis. This is a macerated segment of femur showing a thick layer of sub periosteal new bone. Congenital syphilis classically gives rise to both a florid periostitis (with reactive new bone formation), as in this example , or an osteochondritis (granulomatous inflammation at the ends of long bones). Similar appearances may be seen in tertiary acquired syphilis . in which coexistent gummata may also be present.
Fig.12.8 Ric k.,. throuqlrllr,' I, 'WI'I 10 uppel 111".1, ,1 , , 1'110 , markeclllll( 1" '"1110 1' plat es ,'"1 I .r ' .1' 11111" endochl,,"I/ ,11 1 II, I Rick ets FII II I ! ,'.1, ,, 'It I both c h; II 111 1"1 11 " " I I bon e rnllll :I. II,· .. 011 1" is a dis8;'1,.I' ,'I I 1111 1II compl Clloll l ll'lif llN l l latter IS ~;l'O II III . II II ,II comrTlo r)(,H " I
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Fig.12.7 Osteoporosis. Thi s macerated section of the lower thoracic and lumbar vertebrae shows a very marked reduction in the amount of cancellous bone associated with a crush fracture . Osteoporosis, defined simply as a decrease in volume of other wise normal bone, is pre dominantly a disease of the elderly (particularly females) . Causes , other than ageing or the menopause. include prolonged immobility, disuse, Cushing 's disease or thyrotoxicosis , but most cases remain idiopathic . The spine and pelvis are most often affected and the principal complication is a fracture.
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95
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12 Osteoarticular System Fig.12.6 Syphilitic periostitis. This is a macerated segment of femur showing a thick layer of sub periosteal new bone. Congenital syphilis classically gi ves rise to both a florid periostitis (with reactive new bone formalion). as in this example, or an osteochondrilis (granulomatous inflammation at the ends of long bones). Similar appearances ma y be seen in tertiary acquired syphilis. in which coexistent gummata may also be present.
Fig.12.8 Rickets. Thi s section through the lower femur and upper tibia of a child shows marked thickening of the growth plates and a significant failure of endochondral calcif ication. Rickets and osteomalacia are both characterised by failure of bone mineralisation ; the former is a disease of children (prior to completion of growth) . while the latter is seen in adults . The commonest cause is diminished serum vitamin D which may be due to dietary deficiency. in sufficient sun exposure, malab sorption. chronic renal disease or chronic liver disease . Com plications include bony deformities and fractures.
,
1
Fig.12.7 Osteoporosis. This macerated section of the lower thoracic and lumbar vertebrae shows a very marked reduction in the amount of cancellous bone associated with a crush fracture. Osleoporosis, defined simply as a decrease in volume of other wise normal bone, is pre dominantly a disease of the elderl y (particularl y females) . Causes, other than ageing or the menopause. include prolonged immobility, disuse. Cushing's disease or thyrotoxicosis, but most cases remain idiopathic The spine and pelvis are most often affected and the principal complication is a fracture.
Fig.12.9 Tophaceous gout. This segment of tendon IS covered by pale. Irregular excrescences (tophi) , composed of urate crystals. Gout may be primary, due to an Idiopathic disorder of purine metabolism . or secondary, due either to failure of renal urate excretion or excessive turnover of nucleic acids (e.g . in malig nant disease'or cytotoxic therapy). The consequent hyperuricaemia results In deposition of monosodium urate crystals in jOints, periarticular or subcutaneous tissues. Compli cations include the formation of urate stones in the urinary tract and the deposition of urate crystals in the renal tubules, sometimes leading to renal failure .
Fig.12.10 Hyper parathyroidism (Osteitis fibrosa cystica). A coronat section through the symphysis pubis shows irregular resorption of the para'articular cortical bone and adjacent hyperaemia. Hyperparathyroidism may be primary, secondary or tertiary (see Chapter 7) Up to 25% of affected patients develop bony changes . characterised by Increased bone resorption . medullary fibrosis and the formation of 'brown tumours'. whi ch are haemorrhagic foci of fibrosi s with cyst formation. In cases secondary to chronic renal failure the features may be modified by coexistent osteomalacia. Fig.12.11 Avascufar necrosis. This irregular femoral head shows a large. poorl y'circumscribed area of yellowish necrosis, associated with bony collapse Avascular necrosis (osseous infarction), which occurs pre dominantly in the femoral head, most often follows a sub· capital fracture . It may also occur in decompression sickness. in patients on steroid therapy. in alcoholics or following renal transplantation. Even though the articular cartilage is spared , micro fractures and collapse of the affected bone often lead to arthritis .
96
12 Osreoanicuiar System
Fig.12.13 Rheumatoid arthritis. The femoral condyles , tibial head and patella show very extensive cartilaginous destruc· tion, particularly at the periphery of the articular surfaces . Osteophytes are absent. Rheumatoid arthritis is a chronic systemic inflammatory disease which is commonest in the 3rd to 5th decades and shows a predilection for females . Small joints are principally affected, usually symmetrically, but progressive involvement of large joints is common. The condition is thought to be autoimmune in nature, is more frequent in patients with HLA·DW4 and is associated with the presence of a serum immunoglobulin known as the rheumatoid factor .
Fig.12.12 Osteoarthrosis. This femoral head (top) is irregular in outline, shows eburnation of its superior aspect and also irregular cystic defects at the sites of cartilaginous loss anteriorly. Below , another example seen in cross·section, shows a multilocular focus of cystic degeneration in the subchondral bone associated with marked osteosclerosis and early osteophyte formation laterally. Osteo· arthrosis is an extremely common, non·inflammatory, 'wear and tear' phenomenon, which affects mainly large weighl -bearing joints (usually asymmetrically). Recognised predisposing factors include increasing age, obesity and pre·existent conditions such as congenital hip dislocation, genu varum, Perthes disease or previous fracture . Associaled findings in affected patients include cervical spondylosis, hallux rigidus and Heberden's nodes (osteophytes over the terminal interphalangeal joints).
97
Fig.12.14 Rheumatoid arthritis. This is the synovium from a knee joint, which shows florid synovial villous hyperplasia (pannus for· mation) Chronic synovial inflammation with hyperplasia are the cardinal features of this disease and precede destruction of the articular cartilage. Systemic features of rheumatoid arthritis may include subcutaneous rheumatoid nodules , splenomegaly, Caplan's syndrome and secondary amyloidosis . Pathologically, the arthritis associated with psoriasis and ulcerative colitis is almost indistinguishable.
Fig.12.15 AllkV" 1 spondylltill. /I ' ., ' , th ese 11,,,,1 1. 11 VI'll , I ,
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12 Osteoarticular System Fig.12.15 Ankylosing spondylitis. A section through these lumbar verte brae shows widespread osseous fusion throug h th e intervert ebral discs, resulting in ankylosis. Ankylcsing spondylitis is an idiopathic dis order, whic h occurs most often in young men and is strong ly associated with HLA-B27 . The disease is charact erised by features similar to those of rheumatOid arthriti s, except for the addition of articular ossific ation. It aff ects pre domi nantl y the axial skeleton. Comp lica tions include seve re kyphosis, sometimes with res plratoryembarra ssment.
Rheumatoid femoral condyles , patella show ve ry ilaginous destruc rly at the periphery surfaces . re absent.
and shows a for females. Small ipallyaffec ted , rically, but invol vement of large on . The condition
Fig.12.17 Paget's disease. This proximal portion of femur, whil e showing simil ar featu res to Fig . 12.16, also demonstrates residual foci of porotic bone and typical marked hype raemia. Th is latt er, acting as a multifocal arteriovenous shunt. may give rise to congestive ca rd iac failure . Other complications of Pag et's d isease inc lude bony deformi ty, a predisposition to fractures (th e thick but irregular new bone is weaker than normal) and the development of osteosarcoma or c hondro sarcoma in 1 % of cases .
,. Fig.12.16 Paget's disease (Osteitis deformans). This segment of macerated femur shows marked, irregutar thickeni ng of the co rtica l bone and replacement of cance ll ous bon e by coarse trabecu lae . Pag et's disease, which is thought to be due to a sl ow viral infection, affects up to 2% of th e population (usually subclinically) and shows a predilection for older adults. It is c haracterise d initially by excessive bone resorption and latterly by a marked increase in irregular new bone fo rmat ion. The axia l skeleton , particu larly the spine and skull, is most often affected althou gh the long bones are commonly involved .
Fig .12.18 Charcot's joint. This corona l se ction through a knee jOint demonstrates gross distortion by subluxation and des tructi on of the arti cular cartilage These features have developed as a complication of neurological disease associa ted with loss of pain sensation or proprioception . Classical ca uses include tabes dorsali s, peripheral neuropathy (often diabetic) and syringomye lia.
ritis may , Caplan's ca lly, the arthritis almost
98
12 Osteoarticular System Fig.12.19 Hyper ostosis frontalis interna. Projec tin g from the inner surface o f the frontal bones is a we ll circumscribed mass of extensi vely ridged. rather greyish bone. Hyperostosis frontalis interna is a not uncommon idio pathic le sion, which is usually seen in late adulthood and is rather more common in women. It is rarely of any clin ical sig nificance . Localised reactive hyper ostosis of the skull may also sometim es be seen overlyi ng a meningi oma
Fig.12.20 Fibrous dysplasia. Thi s segmen t of rib is markedly expanded b y a well ci rc umscribed , pale mass . Fibrous dysplasia is regarded as a hamartomatous lesion, composed of fibrous tissue and woven bone, and may take three forms : (1) the monostotic variant (commonest), which is seen at any age and usually affect s long bones or the ribs; (2) the polyostoti c variant, which typically. present s in c hild hood, is often unilateral and may also affect the sku ll ; and (3) the polyostotic variant as sociated with cafe-au-Iait spots and end oc rine abno rmalities (Albright' s synd rome). Sarcomatous change occurs in about 1 % of cases.
99
Fig.12.21 Ivory osteoma . Projecting from the superior surfac e of thiS skull is a smooth, rounded nodule. Ivory osteomas are benign tumours, composed of densely sclerotic mature bone, which usually arise only from the sku ll or facial bones. They may present at any age, show a slight predi lec tion for males and never undergo malignant change. They are some times a feature of Gardner's syndrome (familial polyposis co li with epidermoid cysts, fibromatoses and bone tumours)
Fig.12.22 Osteoid osteoma. Arising from the co rtex of thi s segment of bone is a well circumscribed, vascular nodule . There is adjacent bony sclerosis . Os teoid os teomas are benign tumours which present most often in childhood or adolescence, are commoner in males and are typically very painful (espec ially at night). They arise pre dominantly in the shaft of long bones, particularly the leg , and class ically the associated pain is relieved by aspirin . They do not undergo malignant c hange.
Fig.12.23 Cartilage-capped exostosis. 11,1: , " ", I' ii, V.I projected from the surface of a femur , S II OW~ " I JlIII.·, 'III cartilage overlying a nodule of cortical bOllu II " ", " " II I known as os teochondromas o r ecchondro' lIil'.i) .,,,, 11 ,1]0 deve lopmental le sions, derived Irom lalc r:llly ,Il l''''. "", ca rtil age, wh ic h then undergoes endoc honrll , II, ".·.iI,' . present mos t often in the lower femur or IIPI" " Iii ,I.' "I , I young adult s. Rarely, they may be mult iple (:'111111 " " Ii " , I known as diaphyseal ac lasis), in whic h Clrc, 1111' ,1.1 111 " II" 20 % risk of developing chondrosarco rJ'l ;)
Fig.12.24 rndll"" Thi s CUI (111. Ii ' .• " I" " I 10we r (:llIl " III" . I, .,I' multilJlt ! 11111.' \l II 'Y " carlilacJ" III III,·. "1 "1 1' p h ys l ~: :li ll i , It, '1111 d rollla~: :111 ' I lltlll'"1 1 whic ll lll ol ylli ' '" 011 101' ariSlnq 1111 1", 1. "",1 " yOUII Cj :111 1111'0) 1111111 ' preSCIIGi' "I 1111 iii 'I Ii, (kno wllil:,I )lh. ,, '· ,I , thouqllll< l 11I' 1llIllI, 1 may 11( ' ,1'"lIl' 1,11 .. , 1' t1 55 tH! l': Ii'II " II II II< ", I syn<'lfoll" ') I\II Y 1'>1 1 rntfltl"I.· Ii "." '"' , 110 1 ri!::ik of I II -V I ,I, 'I 111111 i sarCOl1 1.1 t
12 Osteoarticular System
Fig.12.23 Cartilage-capped exostosis. This lesion. which projected from the surface of a femur, shows a pale outer rim of cartilage ove rlying a nodule of cortical bone . These exostoses (also known as osteochondromas or ecchondromas) are thought to be developmental lesions , derived from laterally aberrant epiphyseal ca rtitage, which then undergoes endochondral ossification . They present most often in the lower femur or upper tibia of children or young adults. Rarely, they may be multiple (an inherited condition known as diaphyseal aclasis). in which circumstance there is about a 20% risk of developing chondrosarcoma. Fig.12.24 Enchondromatosis. This coronal section through the lower end of the femur shows multiple blue-grey nodules of cartilage in the epiphysis, meta physis and diaphysis. Enchon dromas are benign tumours, which may be solitary (typically arising in the long bones of young adults) or multiple . The presence of multiple lesions (known as Oilier's disease) is not thought to be hereditary and may be associated with soft tissue haemangiomas (Maffuci's syndrome). Any patient with multiple lesions has a significant risk of developing chondro sarcoma.
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Fig.12.25 Aneurysmal bone cyst. This lesion, removed from the femur, is composed of a well circumscribed haemorrhagic mass within which are numerous vascular spaces. Aneurysmal bone cysts are benign tumours, probably of vascular origin, which typically ari se in the long bones or the spine of adolescents and young adults. They are often painful and tend to local recurrence if in adequately excised . Their occasional coexistence with other adjacent benign tumours of bone further confuses their -.L!l uncertain histogenesis .
Fig.12.26 Osteoclastoma (giant cell tumour of bone). Arising in the epiphysis of this femur and extending into the metaphysis is a reasonably circumscribed. haemorrhagic mass. Giant cell tumours of bone are uncommon lesions which tYP ically present in young adults and tend to arise in the epiphysis 01 long bones (particularly in the leg) . Their histogenesis is uncertain and they must be dis tingUished from other giant cell lesions such as chondroblastoma . chondromyxoid fibroma or hyperparathyroidism . Up to 25% behave in a malignant fashion .
100
12 Osteoarticular System
Fig.12.27 Osteosarcoma. Arising in the metaphysis of this femur is an ill·defined, pale and focally haemorrhagic tumour which has elevated the periosteum and eroded into adjacent soft tissue. Osteo sarcoma. in the majority of cases, presents in the first two decades, shows a predilection for males and classically arises in the metaphysis of a long bone (particularly the femur or tibia). A small proportion of cases are seen in the elderly, secondary to Paget's disease (see Figs. 12.16 and 12.17). They tend to extensive local invasion and early haematogenous spread. the overall 5-year-survival being about 20%
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Fig.12.28 Chondrosarcoma. Arising from the pelvis, adjacent to the acetabulum. is a widely invasive tumour, largely composed of irregularly lobulated, blue-grey carti laginous tissue. In contrast to osteo sarcoma. chondro sarcoma typically presents in the 6th and 7th decades and arises most often in the pelvis (although proximal long bone involve ment is not uncommon). It tends to be slow-growing. often attaining a considerable size.. and 5-year-survival is about 75%
101
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Fig.12.29 Multiple myeloma. This portion of skull (top) shows multiple punched-out lesions containing haemorrhagic tumour. Below, a segment of the spine demonstrates ill demarcated, haemorrhagic, osteolytic lesions in the lower cervical vertebral bodies. Multiple myeloma typically presents in late adulthood and is characterised by a neoplastic proliferation of plasma cells, which classically gives rise to osteolytic lesions in the marrow of the axial skeleton. Excessive immunoglobulin production by the tumour cells allows detection of the light chain Bence-Jones protein in the urine. a useful diagnostic aid. Complications include a predisposition to infection. renal damage and amyloidosis The prognosis is very variable.
12 Osteoarticular System Fig.12.30 Meta static breast carcinoma. Within the vertebral bodies are scattered, pale, soft necrotic nodules of metastatic tumour. Metastases are the commonest tumour to be found in bone and are most often osteolytic in character. While any disseminated malignancy may involve bone, the commonest primary sources responsible are carcinoma of the breast, lung, prostate, kidney and thyroid .
Fig .12.31 Meta static prostatic carcinoma. The vertebral bodies at the base of this spine are largely replaced by whitish , firm, ill-defined metastatic tumour. Secondary prostatic carcinoma in bone classically stimulates local new bone formation thus giving rise to an osteosclerotic appearance. Breast carcinoma may sometimes have a similar effect.
102
l[f[[I[lrr[[[[lf[II[[[II[[--lf[llr[[I~rrr[ Index Achalasia , 25 Acoustic neuroma, 93 Adenocarcinoma breast, 46-49
duodenum, 28
gallbladder, 43
kidney, 68
large bowel , 34-35
lung , 20-21
ovary , 74-75
pancreas , 44
prostate, 85
stomach,27-28
uterUS , 80
Addison 's disease, 59 , 60 Adenomyoma, 79 Adenomyosis , 79 Adrenal gland Addison 's disease, 59 haemorrhage, 59 nodular hyperplasia, 60 tumours, 60-61 Alzheimer'sdisease , 91 Amyloidosis renal,68 spleen, 51 Aneurysmal bone cyst , 100 Aneurysms aortic , 11-12 berry, 90 cerebral artery, 90 dissecting , 11-12 left ventricular, 2 splenic artery, 12 true, classification of, 11 Ankylosing spondylitis, 98 Aorta aneurysms, 11-12 atheroma, 9-1 0 fatty streaks ,9 syphilis, 10 Aortic valve incompetence , causes , 5 infective endocarditis , 5-6 non-infective endocarditis, 6
103
stenosis , 4-5 Aortitis, syphilitic, 5, 10 Appendicitis, acute , 31 Appendix , carcinoid tumour, 30 Asbestos and lung disease , 22 Atheroma, 1, 9 complicated , 10
risk factors , 9
ulcerated , 10
Atrium , left, myxoma of, 8 Barrett's ulcer, 24 Bileducts carcinoma, 43 gallstones in , 42 Bladder diverticulum , 71 transitional cell carcinoma, 71 Bones fibrous dysplasia, 99 necrosis, 96 tumours, 99-102 see a/so Fractures and specific disorders of bones Bowel, large amoebic dysentery, 33 chronic ischaemic colitis, 32 Crohn 's disease , 29 diverticular disease, 32 diverticulitis , 32 pseudomembranous colitis , 33 tumours, 33-35 ulcerative colitis , 31-32 Bowel , small Crohn's disease , 29 infarction, 30 ischaemia, 30 tuberculosis, 30 tumours, 30 typhoid infection, 29 ulcers, 29 Bowen 'sdisease, vulval, 82 Brain abscess , 88 atrophy, 91
fat embolism , 89
general paresis, 89
haemorrhages, 89-90
hydrocephalus, 87
infarction , 91
meningitis, 88
raised intracranial pressure, 89
tumours, 92-93
Breast carcinoma, 46-49 duct papillomata, 46 fibroadenoma, 45-46 fibrocystic disease, 45 gynaecomastia,49 male, 49 mammillary fistula, 45 non-Hodgkin 's lymphoma , 49 peau d'orange , 48 Brenner tumour, 75 Bronchi , tumours, 20-21 Bronchiectasis , 15,21 causes , 15 Bronchopneumonia , 13, 15 with bronchial carcinoma, 21 tuberculous , 16 Budd-Chiari syndrome, 38 Burkitt's lymphoma, 52 Caecum, carcinoma of, 35 Calculi bile duct, 42 gallbladder, 42 , 43 renal , 64 stag horn , 64 Candidiasis , oesophageal , 24 Carcinoma see sites of carcinoma Cardiomyopathy congestive, 7 hypertrophic obstructive , 6 types of, 6 Cerebral see Brain Cerebral artery , aneurysms , 90 Cerebrospinal fluid , normal circulation, 87 CerebrovaSCular accident, causes, 90, 91
Charcot's joint, 98 Chemodectoma, 61 Cholangiocarcinoma,43 Cholecystitis acute, 42 chronic, 42 Cholelithiasis, 42, "3 Cholesterolosis , 43 Chondrosarcoma, 100, 101 Choriocarcinoma testis , 84 uterus , 81 Cirrhosis, 39-40 Coalminers, lung diseases, 17-18 , 1!) Colon/Colitis see Bowel, large Conn's syndrome, 60 Coronary artery thrombosis, 1 Corpora albicantia, 72 Corpus luteum , 72 Craniopharyngioma , 55 Crohn 's disease, 29 Cushing 's syndrome , 60 Cystadenocarcinoma (ovary) mucinous, 75 serous, 74 Cystadenoma (ovary) mucinous , 74 serous, 73 Duke's staging of large bowel tumOllr~: . : 1'1 Duodenum periampullary carcinoma, 28 ulcers , 28 Dysentery, amoebic, 33 Dysgerminoma, 76
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11,,1 , il',1 I 'illi ll ) II.
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Cholangiocarcinoma,43
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acute, 42
chronic, 42
Cholelithiasis, 42, .13
CholesterolosiS,43
Chondrosarcoma, 100,101
Choriocarcinoma
testis, 84
uterus, 81
Cirrhosis, 39-40
Coalminers, lung diseases, 17-18, 19
Colon/Colitissee Bowel, large
Conn'ssyndrome, 60
Coronary artery thrombosis, 1
Corpora albicantia, 72
Corpus luteum, 72
Craniopharyngioma, 55
Crohn's disease, 29
Cushing's syndrome, 60
Cystadenocarcinoma (ovary)
mucinous, 75
serous, 74
Cystadenoma (ovary)
mucinous, 74
serous, 73
Duke's staging of large bowel tumours, 35
Duodenum
periampullary carcinoma, 28
ulcers, 28
Dysentery, amoebic, 33
Dysgerminoma, 76
Ecchondromas, 100 ,
Ectopic pregnancy, 77
Emphysema
centriacinar, 19
classification, 19
focal dust, 19
panacinar,19
paraseptal,20
Enchondromatosis, 100
Endoci Irdill•. aculOdl(Jlll llllli. , I ·1 infe(;tivu, ~ , non-lf1feCliv" II". ",il lI/ Iii: , () types or , ~ , Endocrine NI '''II IiI'd, I : 'Y 'IIII' l1Tl O , Multiple see Mulli plt l I 11I 1"l lil l' I N' H!plasia Synd"""" Endometri0111c: ovarian, 7: \ Endometriulll
adenocar,:11 11 1111(1.1\( I
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polyp, 79
Endomyoca rdl lllll, 1,1,14 1'11:1 , /
EpendymOlTlu , Oil
Epicardium, tllulll(\llIlil ' dUpOSltS, 8
Epididymitis
abscess,8!)
chronic, 85
tuberculous, 8
Exostosis, carlil ago cappeu, 100
Fallopian tubes
abscess,72
cysts, 77
ectopic pregnancy, 77
hydrosalpinx, 78
inflammation, 72, 78
pyosalpinx, 78
Fatty change
heart, 9
liver, 38-39
Fibroids,79 Fractures, 94
malunion, 94
non-union with false joint, 94
osteoporosis and, 95
Gallbladder
carcinoma, 43
cholecystitis, 42
cholesterolosiS,43
stones, 42
Gastritis
acute, 26
chronic atrophic, 27
Ghon focus, 15
Glioblastoma multiforme, 92
Gliomas, 92
Glomerulonephritis
acute proliferative, 66
chronic, 67
membranous, 66
Glomus jugulare tumours, 61
Goitre
colloid, 56
diffuse toxic, 56
multinodular, 56, 57
Gout, tophaceous, 96
Granulosa cell tumour, 75
Graves' disease, 56
Grawitztumour, 68
Gynaecomastia,49
Haemochromatosis, 39, 40
Haemopericardium, 2,12
Haemorrhages
intracerebral,90
pontine, 90
subarachnoid, 90
subdural, 89
Haemosiderosis, pulmonary, causes, 17
Hamartoma, pulmonary, 20
Hashimoto'sdisease, 56, 57
Heart
brown atrophy, 9
see a/so specific parts and diseases of
heart
Hodgkin's disease
breast, 49
hepatic involvement, 41
lymph nodes, 53
spleen in, 52
Honeycomb lung, 18
Hydatid cyst, hepatic, 37
Hydatidiform mole, 81
Hydrocephalus, 87, 89
causes, 87
Hydronephrosis, 68, 69, 70, 71
104
Hydrosalpinx, 78 Hyperaldosteronism, 60 Hyperostosis frontalis interna, 99 Hyperparathyroidism, 58, 96 Hypothyroidism, 56, 57 Infarct bone,96 cerebral,91 hepatic, 37 intestinal , 30 myocardial, 1, 2 pulmonary, 17 renal,65 testicular, 83 Intestines see Bowel Islet cell tumours, 44 Joints, 96, 97, 98 Kidney acute pyelonephritis , 63 amyloidosis, 68 chronic interstitial nephritis , 64 cortical lobulation, 62 cortical necrosis , 65 cyst, simple, 63 dysplasia, 63 essential hypertension, 66 glomerulonephritis, 66-67 horseshoe, 62 hydronephrosis, 68, 69 , 70, 71 infarction , 65 lobulation , 62 medullary sponge, 63 nephrotic syndrome, causes of, 67 papillary necrosis, 68 polycystic, 62 renal vein thrombosis, 65 stones, 64 transplant rejection, 65-66 tuberculosis, 64 tumours, 68-69 Krukenberg tumour, 77
105
Lambl's excrescence , 8 Larynx, carcinoma, 13 Leiomyoma gastric, 28 uterine , 79 Leiomyosarcoma, uterine , 80 Leukaemias , spleen and , 51-52 Leukoplakia, oral , 23 Linitis plastica, 28 Lipids, cardiovascular deposition, 9 Liver abscesses, 36 amoebiasis, 36 amyloidosis, 39 Budd-Chiari syndrome, 38 cirrhosis, 39-40 fatty change , 38-39 hydatid disease , 37 necrosis, 36 polycystic, 36 portal vein thrombosis, 37 tumours, 38, 40-41 venous congestion , 37 Zahn infarct, 37 Lobar pneumonia, 13 Lung abscesses, 14 acini,19 emphysema, 19-20 occupational disease , 17-18,19, 22 sarcoidosis, 51 tuberculosis, 15-16, 18 tumours, 20-22 Lymph node Hodgkin 's disease, 53 malignant melanoma, 54 non-Hodgkin 's lymphoma, 54 sarcoidosis, 53 secondary carcinoma, 54 tuberculosis, 53 Lymphoma adrenal gland, 61 breast, 49 Burkitt 's, 52
hepatic involvement, 41 intestinal , 31 lymph nodes , 53-54 thyroid gland, 58 see also Hodgkin 's disease ; Non Hodgkin 's lymphoma Maffucci syndrome, 100 Malignant melanoma, lymph nodes, 54 Mallory- Weiss tear, 24 Meckel 's diverticulum , 29 Mendelson's syndrome, 14 Meningioma, 92 Meningitis bacterial,88 tuberculous , 88 Mesenteric artery, embolism , 30 Mesothelioma, 22 Metastatic carcinoma adrenal , 60 bone , 102 brain , 93 heart, 8 liver, 41 lung , 21 , 22 ovary, 77 spleen , 53 Miners, lung disease , 17-18, 19 Mitral valve incompetence , causes of, 4 infective endocarditis, 5 mixed disease , 4 non-infective endocarditis, 6 in rheumatic endocarditis, 3 stenosis, 3 Mbnckeberg 's sclerosis, 12 Mouth leukoplakia, 23 squamous carcinoma, 23 Mullerian tumour, malignant mixed , 80 Multiple Endocrine Neoplasia Syndrome, 58 typel,55 type II, 58, 61
Multiple sclerosis, 9 1 Myeloma , multiple, 10 1 Myocardial infarct, 1, 2 causes , 1 complications, 2 Myocardial rupture , 2 Myocardium brown atrophy, 9 fatty degeneration , 9 Myxoedema, 57 Myxoma, left atrial , 8 Nabothian cysts, 79 Nephritis , chronic interstitial , 64 Nephroblastoma,69 Nephrotic syndrome , causes of, 67 Neurilemmoma, 93 Neuroblastoma, 61 Neurofibroma, plexiform , 93 Neuroma, acoustic, 93 Nipple Paget's disease, 48 retraction , 47 Non-Hodgkin 's lymphoma adrenal gland, 61 breast, 49 Burkitt's, 52 hepatic involvement, 41 , 52 intestines , 31 lymph nodes , 54 spleen , 52 thyroid gland , 58 Oesophagus achalasia , 25 candidiasis , 24 carcinoma, 25 Mallory- Weiss tear, 24 peptic ulcer, 24 stricture, 24 varices, 25 Oilier's, disease, 100 Oophoritis, pyogenic, 72 Osteitis
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Multiple sclerosis , 91
Myeloma, multiple, 101
Myocardial infarct, 1, 2
causes, 1
complications, 2
Myocardial rupture, 2
Myocardium
brown atrophy, 9
fatty degeneration , 9
Myxoedema,57
Myxoma , left atrial, 8
Nabothian cysts, 79
Nephritis, chronic interstitial, 64
Nephroblastoma,69
Nephrotic syndrome, causes of, 67
Neurilemmoma, 93
Neuroblastoma, 61
Neurofibroma, plexiform , 93
Neuroma, acoustic, 93
Nipple
Paget's disease , 48
retraction , 47
Non-Hodgkin 's lymphoma
adrenal gland, 61
breast, 49
Burkitt 's, 52
hepatic involvement, 41 , 52
intestines, 31
lymph nodes, 54
spleen , 52
thyroid gland , 58
Oesophagus
achalasia , 25
candidiasis, 24
carcinoma, 25
Mallory-Weiss tear, 24
peptic ulcer, 24
stricture, 24
varices, 25
Oilier's, disease, 100
Oophoritis , pyogenic , 72
Osteitis
deformans, 98
fibrosa cystica, 96
Osteoarthrosis,97
Osteochondritis , syphilitic , 95
Osteochondromas, 100
Osteoma
ivory , 99
osteoid , 99
Osteomalacia, 96
Osteomyelitis
chronic, 95
tuberculous, 95
Osteoporosis, 95
Osteosarcoma , 101
Ostoclastoma, 100
Ovary
abscess , 72
corpora albicantia , 72
corpus luteum, 72
cystic follicles , 72
endometriosis, 73
torsion , 77
tumours, 73-77
Paget 's disease
ofbone,98
of nipple, 48
Pancreas
cysts, 44
tumours, 44
Pancreatitis
acute, 43
chronic, 44
Papillary muscle, rupture of, 2, 4
Parathyroid glands
hyperplasia, 58
tumours, 58
Pelvi-ureteric junction obstruction, 70
Pelvis, renal, transitional cell carcinoma,
69
Penis, carcinoma , 86
Pericarditis
constrictive, 8
fibrinous, 1, 7,8
tuberculous, 7
Periostitis, syphilitic, 95
Perisplenitis, chronic, 50
Phaeochromocytoma , 61
Pharyngeal pouch , 23
Phyllodes tumour, 46
Pituitary tumours, 55
Pleomorphic adenoma, parotid , 23
Pleura
hyaline plaques, 22
mesothelioma , 22
Plummer-Vinson syndrome , 25
Pneumoconiosis, 17
Pneumonia
aspiration , 14
broncho- , 13, 15, 16, 21
Klebsiella, 14
lipid , 14
10':>ar,13
staphylococcal , 14
Polyarteritis nodosa, 10
Polycystic disease
kidney, 62
liver, 36
Polyposis coli, familial, 34
Polyps
breast, 46
cervix, 81
endometrium, 79
large bowel, 33-34
stomach,27
Portal vein, thrombosis, 37
Polt's disease , 95
Pregnancy, ectopic, 77
Prostate gland
benign hypertrophy, 86
carcinoma, 85
Pulmonary
embolism , 16
haemosiderosis, 17
infarct, 17
Pyelonephritis, acute, 63
Pylorus , congenital stenosis, 25
Pyonephrosis, 64
106
Pyosalpinx, 78 Rectum
tumours, 34-35
ulcerative colitis, 31
Renal vein thrombosis , 65
Rheumatic heart disease , 3-5
Rheumatoid arthritis , 97
Rickets, 96
Salivary glands, mixed tumour, 23
Salpingitis
acute, 72, 78
chronic, 78
Sarcoidosis
lymph nodes, 53
spleen , 51
Schwannoma , benign, 93
Seminoma, 84
Silicosis, 18
Spermatic cord, torsion, 83
Spinal cord, ependymoma, 92
Spine, tuberculosis, 95
Spleen
amyloidosis, 51
'cricket ball', 50
enlargement, 50, 51 , 52
Hodgkin's disease, 52
infarction , 50
leukaemias and, 51-52
myelofibrosis, 51
non-Hodgkin 's lymphoma, 52
passive venous congestion, 50
sarcoidosis , 51
secondary carcinoma, 53
'sugar-icing ', 50
tuberculosis, 51
Splenic artery aneurysm, 12
Squamous carcinoma
cervix , 82
larynx, 13
lung,20
oesophagus, 25
penis, 86
vagina, 82
107
vulva, 82 Stomach
gastritis, 26, 27
'leather-bottle ', 28
pyloric stenosis , 25
tumours, 27-28
ulcers, 26-27
Stroke , causes, 90 , 91
Struma ovarii , 76
Syphilis
and aortitis, 5, 10
and general paresis, 89
and periostitis, 95
Syringomyelia, 87
Teratoma
differentiated,84
malignant intermediate (MTI), 84
malignant trophoblastic (MTT) , 84
mature cystic, 76
ovarian, 76
testicular, 84
Testis
atrophy, 83, 85
torsion , 83 , 84
tumours, 83-84
Thecoma, 76
Thrombosis
coronary artery, 1
deep venous, 11
portal vein , 37
Thrombus, mural, 1,2
Thymoma, 54
Thyroglossal cyst, 55
Thyroid gland
carcinoma, 57-58
enlargement, classification, 55
gOitre, 56
Graves' disease, 56
Hashimoto's disease, 56
lymphoma, ?8
myxoedema,57
Tongue, carcinoma, 23
Tuberculosis
adrenal gland , 59
epididymis , 85
heart, 7, 8
intestinal , 30
lymph nodes, 53
and meningitis , 88
miliary, 16, 51
pulmonary , 15-16, 18
renal,64
spinal,95
spleen, 51
Tumours see sites and specific types of
tumour
Typhoid, small intestine, 29
Ulcerative colitis, 31-32
Ureter
duplication of, 69
transitional cell carcinoma, 70
ureteritis cystica , 70
Urethral valve , 71
Uterus
bicornuate, 79
cervical carcinoma, 82
cervical 'erosion ', 81
endocervical polyp , 81
tumours , 79-81
see also Endometrium
Vagina, carcinoma, 82
Ventricle,lef1
aneurysms, 2
hypertrophy, 3, 4, 6
Vulva
Bowen'sdisease, 82
carcinoma, 82
Waterhouse--Friderichsen syndrome, 59
Wilm's tumour, 69
