Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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Multilocular cystic renal cell carcinoma J.N. <strong>Eble</strong> Definition A tumour composed entirely of numerous cysts, the septa of which contain small groups of clear cells indistinguishable from grade Ι clear cell carcinoma. ICD-O code 8310/3 Clinical features There is a male:female predominance of 3:1. All have been adults (age range 20- 76 years, mean = 51) {650}. No instance of progression of multilocular cystic renal cell carcinoma is known. Macroscopy While cysts are common in clear cell renal cell carcinomas, only rarely is the tumour entirely composed of cysts. In these tumours the number of carcinoma cells is small and diagnosis is challenging {1835}. In order to distinguish these tumours with excellent outcomes from other clear cell carcinomas, ones containing expansive nodules of carcinoma must be excluded and diagnosed simply as clear cell renal cell carcinoma {650}. Multilocular cystic renal cell carcinoma consists of a well-circumscribed mass of small and large cysts filled with serous or haemorrhagic fluid and separated from the kidney by a fibrous capsule. Diameters have ranged from 25 mm to 130 mm. More than 20% have calcification in the septa and osseous metaplasia occasionally occurs. Tumour spread and staging No tumour with these features has ever recurred or metastasized. Histopathology The cysts are usually lined by a single layer of epithelial cells or lack an epithelial lining. The lining cells may be flat or plump and their cytoplasm ranges from clear to pale. Occasionally, the lining consists of several layers of cells or a few small papillae are present {2561}. The nuclei almost always are small, spherical, and have dense chromatin. The septa consist of fibrous tissue, often densely collagenous. Within some of the septa there is a population of epithelial cells with clear cytoplasm. The epithelial cells resemble those lining the cysts and almost always have small dark nuclei. The clear cells form small collections but Fig. 1.23 Multilocular cystic renal cell carcinoma. do not form expansile nodules. These epithelial cells often closely resemble histiocytes, or lymphocytes surrounded by retraction artefacts. Increased vascularity within the cell clusters is a clue to their nature. Immunoprofile The cells with clear cytoplasm in the septa frequently react strongly with antibodies to cytokeratins and epithelial membrane antigen and fail to react with antibodies to markers for histiocytes. A B Fig. 1.24 Multilocular cystic renal cell carcinoma. A The septa of multilocular cystic renal cell carcinoma contain eptihelial cells which can be mistaken for lymphocytes. B The epithelial cells in the septa of multilocular cystic renal cell carcinoma react with antibodies to epithelial markers. EMA expression. 26 Tumours of the kidney
Papillary renal cell carcinoma B. Delahunt J.N. <strong>Eble</strong> Definition A malignant renal parenchymal tumour with a papillary or tubulopapillary architecture. ICD-O code 8260/3 Epidemiology Papillary renal cell carcinomas (PRCC) comprise approximately 10% of renal cell carcinoma in large surgical series {584,1860}. The age and sex distribution of PRCC is similar to clear cell renal cell carcinoma with reported mean age at presentation and sex ratio (M:F) for large series ranging from 52-66 years and 1.8:1 to 3.8:1, respectively {76,584,587, 1612}. Clinical features Signs and symptoms are similar to clear cell renal cell carcinoma {1612}. Radiological investigations are non-specific, although renal angiography studies have shown relative hypovascularity for PRCC {1860}. Macroscopy PRCC frequently contains areas of haemorrhage, necrosis and cystic degeneration, and in well-circumscribed tumours an investing pseudocapsule may be identified {76,1612}. Bilateral and multifocal tumours are more common in PRCC than in other renal parenchymal malignancies and in hereditary PRCC up to 3400 microscopic tumours per kidney have been described {1979,2169}. Histopathology PRCC is characterized by malignant epithelial cells forming varying proportions of papillae and tubules. Tumour lined cysts with papillary excrescences may also be seen {585,1612,1860}. The tumour papillae contain a delicate fibrovascular core and aggregates of foamy macrophages and cholesterol crystals may be present. Occasionally the papillary cores are expanded by oedema or hyalinized connective tissue {584,585}. Solid variants of PRCC consist of tubules or short papillae resembling glomeruli {585,2173}. Necrosis and haemorrhage is frequently seen and haemosiderin granules may be present in macrophages, stroma and tumour cell cytoplasm {1612}. Calcified concretions are common in papillary cores and adjacent desmoplastic stroma, while calcium oxalate crystals have been reported {587,641,1612}. Two morphological types of PRCC have been described {585}: Type 1 tumours have papillae covered by small cells with scanty cytoplasm, arranged in a single layer on the papillary basement membrane. Type 2 tumour cells are often of higher nuclear grade with eosinophilic cytoplasm and pseudostratified nuclei on papillary cores. Type 1 tumours are more frequently multifocal. Sarcomatoid dedifferentiation is seen in approximately 5% of PRCC and has been associated with both type 1 and type 2 tumours {585}. Immunoprofile Cytokeratin 7 (CK 7) expression has been reported for PRCC {831} however, this is more frequently observed in type 1 (87%) than type 2 (20%) tumours {585}. Ultrastructural findings are not diagnostic and are similar to clear cell renal cell carcinoma {1888,2609}. Grading There is no specific grading system for PRCC and the Fuhrman system {815} is accepted as applicable to both clear cell renal cell carcinoma and PRCC. Table 1.04 Immunohistochemical profile of PRCC. Antibody Number % showing of cases positive expression AE1/AE3 36 100 CAM 5.2 11 100 EMA 11 45 Vimentin 116 51 S-100 11 55 Callus 36 92 34βE12 36 3 CEA 36 11 RCC 14 93 CD-10 14 93 Ulex europeaus 105 0 ________ From {140,585,831,1693,2169}. A B Fig. 1.25 Papillary renal cell carcinoma. A The papillary architecture is faintly visible in the friable tumour. B Gross specimen showing tumour haemorrhage and pseudoencapsulation. C Yellow streaks reflect the population of foamy macrophages. C Multilocular cystic renal cell carcinoma / Papillary renal cell carcinoma 27
Page 1 and 2: World Health Organization Classific
Page 3 and 4: This volume was produced in collabo
Page 5 and 6: Contents 1 Tumours of the kidney 9
Page 7 and 8: CHAPTER 1 Tumours of the Kidney Can
Page 9 and 10: TNM classification of renal cell ca
Page 11 and 12: one quarter of kidney cancers in bo
Page 13 and 14: Familial renal cell carcinoma M.J.
Page 15 and 16: Table 1.02 Genotype - phenotype cor
Page 17 and 18: A B Fig. 1.11 A Multiple cutaneous
Page 19 and 20: A B Fig. 1.14 Birt-Hogg-Dubé syndr
Page 21 and 22: Clear cell renal cell carcinoma D.J
Page 23: Fig. 1.20 Clear cell renal cell car
Page 27 and 28: A B Fig. 1.29 Papillary renal cell
Page 29 and 30: Fig. 1.33 Chromophobe RCC with sarc
Page 31 and 32: Carcinoma of the collecting ducts o
Page 33 and 34: Renal medullary carcinoma C.J. Davi
Page 35 and 36: Renal carcinomas associated with Xp
Page 37 and 38: Renal cell carcinoma associated wit
Page 39 and 40: Papillary adenoma of the kidney J.N
Page 41 and 42: of this tumour. Microscopic extensi
Page 43 and 44: A B Fig. 1.59 Metanephric adenoma.
Page 45 and 46: esults in intratumoral aneurysms. O
Page 47 and 48: peritumoural fibrous pseudocapsule.
Page 49 and 50: increases in prevalence to approxim
Page 51 and 52: Nephrogenic rests and nephroblastom
Page 53 and 54: Cystic partially differentiated nep
Page 55 and 56: A B Fig. 1.80 Clear cell sarcoma of
Page 57 and 58: A B Fig. 1.85 Rhabdoid tumour of th
Page 59 and 60: transcription factor is fused to th
Page 61 and 62: Leiomyosarcoma S.M. Bonsib Definiti
Page 63 and 64: Angiomyolipoma G. Martignoni M.B. A
Page 65 and 66: melanocytic and smooth muscle marke
Page 67 and 68: Multinucleated and enlarged ganglio
Page 69 and 70: Haemangioma P. Tamboli Definition H
Page 71 and 72: Fig. 1.107 Juxtaglomerular cell tum
Page 73 and 74: Intrarenal schwannoma I. Alvarado-C
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Mixed epithelial and stromal tumour
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Synovial sarcoma of the kidney J.Y.
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Renal carcinoid tumour L.R. Bégin
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Primitive neuroectodermal tumour (E
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Paraganglioma / Phaeochromocytoma P
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Leukaemia A. Orazi Interstitial inf
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WHO histological classification of
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TNM classification of carcinomas of
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The risk of bladder cancer goes dow
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Tumour spread and staging Urinary b
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feature in such patients undergoing
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A Fig. 2.12 Infiltrative urothelial
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A Fig. 2.15 A Infiltrating urotheli
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A B Fig. 2.19 A Infiltrative urothe
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Fig. 2.23 Infiltrative urothelial c
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ing systems have been proposed on t
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Non-invasive urothelial tumours G.
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chronically inflamed urothelium and
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Inverted papilloma G. Sauter Defini
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muscle invasive disease, but there
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Fig. 2.42 Non-invasive urothelial n
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A Fig. 2.45 Non-invasive urothelial
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for DBCCR1 silencing {984,2476}. Th
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Squamous cell carcinoma D.J. Grigno
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Fig. 2.51 Squamous cell carcinoma.
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Adenocarcinoma A.G. Ayala P. Tambol
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A B Fig. 2.61 A Adenocarcinoma in s
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A B Fig. 2.65 Intramural urachal ca
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Müllerian origin is postulated for
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A Fig. 2.69 Small cell carcinoma. A
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Carcinoid L. Cheng Definition Carci
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Leiomyosarcoma J. Cheville Definiti
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Osteosarcoma L. Guillou Definition
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Leiomyoma J. Cheville Definition A
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Haemangioma L. Cheng Definition Hae
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Metastatic tumours and secondary ex
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Tumours of the renal pelvis and ure
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nodular, ulcerative or infiltrative
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Tumours of the urethra F. Hofstädt
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usually show enteric, colloid or si
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CHAPTER 3X Tumours of of the the Pr
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TNM classification of carcinomas of
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contrast, mortality among migrants
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Fig. 3.06 Transrectal ultrasound of
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PSA-related diagnostic strategies.
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A B C Fig. 3.10 A,B Section of pros
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pale-clear, similar to benign gland
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A B Fig. 3.20 A, B Adenocarcinoma w
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prostate adenocarcinomas exhibit AR
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A Fig. 3.27 A, B Foamy gland adenoc
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A B Fig. 3.32 A Sarcomatoid carcino
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A B Fig. 3.37 A Gleason score 1+1=2
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A B Fig. 3.43 A Prostate cancer Gle
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Fig. 3.48 Heat map-nature. From S.M
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Fig. 3.51 Prostate cancer. Major su
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many stage T1b cancers. Stage T2 Mo
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Fig. 3.58 Patterns of seminal vesic
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Prostatic intraepithelial neoplasia
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A B Fig. 3.63 A Micropapillary high
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A B Fig. 3.68 A Ductal carcinoma in
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Ductal adenocarcinoma X.J. Yang L.
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Papillary pattern can be seen in bo
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A B Fig. 3.74 A Inflammation withou
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Squamous neoplasms T.H. Van der Kwa
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Neuroendocrine tumours P.A. di Sant
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Mesenchymal tumours J. Cheville F.
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Fig. 3.89 Sarcoma of the prostate.
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Miscellaneous tumours P.H. Tan L. C
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A Fig. 3.96 A Adenocarcinoma of the
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Page 215 and 216:
Introduction F.K. Mostofi I.A. Sest
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wartime birth cohorts illustrate th
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Similar tumours as those of group 1
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crucial for the development of this
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A B Fig. 4.09 Spermatocytic seminom
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A B Fig. 4.14 Intratubular germ cel
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A B Fig. 4.19 Seminoma. A Typical s
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Fig. 4.24 Seminoma. Vascular invasi
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Fig. 4.28 Spermatocytic seminoma wi
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A B Fig. 4.33 Embryonal carcinoma.
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A B Fig. 4.38 Yolk sac tumour. A En
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have cytoplasmic lacunae that conta
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A Fig. 4.46 Teratoma. A Longitudina
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A B Fig. 4.51 A Cut surface of derm
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Fig. 4.54 Mixed germ cell tumour. L
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Sex cord / gonadal stromal tumours
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A B C Fig. 4.67 Malignant Leydig ce
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A Fig. 4.73 A Sertoli cell tumour.
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ICD-O codes Granulosa cell tumour 8
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ICD-O code 8592/1 Clinical features
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A B Fig. 4.83 Germ cell-sex cord/go
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A Fig. 4.85 A, B Brenner tumour of
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typical grade III follicular morpho
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testicular parenchyma and tumour te
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A B the surgical scar and adjacent
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Immunoprofile Ordóñez and associa
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often have a grey-white cut surface
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Fig. 4.111 Angiomyofibroblastoma-li
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A B Fig. 4.119 Embryonal rhabdomyos
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Table 4.05 Secondary tumours of the
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Page 277 and 278:
A Fig. 5.04 A, B Squamous cell carc
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deformed by an exophytic mass. In s
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A B C Fig. 5.12 A Warty (condylomat
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A Fig. 5.20 Bowenoid papulosis. A,
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three had been circumcized by 9 yea
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Mesenchymal tumours J.F. Fetsch M.
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Fig. 5.31 Neurofibroma of the penis
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oectodermal tumour/Ewing sarcoma [p
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Secondary tumours of the penis C.J.
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Dr John N. EBLE* Dept. of Pathology
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Dr Ricardo PANIAGUA Department of C
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Source of charts and photographs 1.
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References 1. Anon. (1955). Case re
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115. Ariel I, Sughayer M, Fellig Y,
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246. Birt AR, Hogg GR, Dube WJ (197
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374. Cardone G, Malventi M, Roffi M
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502. Corti B, Carella R, Gabusi E,
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633. Donhuijsen K, Schmidt U, Richt
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768. Fischer J, Palmedo G, von Knob
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900. Goedert JJ, Cote TR, Virgo P,
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1028. Hartmann A, Dietmaier W, Hofs
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1162. Iezzoni JC, Fechner RE, Wong
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1293. Keetch DW, Catalona WJ (1995)
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1424. Ladanyi M, Lui MY, Antonescu
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1548. Lopez-Beltran A, Croghan GA,
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1681. McLaughlin JK, Silverman DT,
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1816. Moudouni SM, En-Nia I, Rioux-
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1945. Ohori M, Wheeler TM, Kattan M
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2070. Phillips G, Kumari-Subaiya S,
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2199. Riou G, Barrois M, Prost S, T
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2327. Schmidt L, Junker K, Weirich
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2450. Smith G, Elton RA, Beynon LL,
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2572. Tamboli P, Mohsin SK, Hailema
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2693. van Echten J, Timmer A, van d
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2816. Wick MR, Berg LC, Hertz MI (1
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2937. Zhuang Z, Park WS, Pack S, Sc
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CCNB, 226 CCND1, 105, 108 CCND2, 22
Page 351 and 352:
J Juvenile type granulosa cell tumo
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Promoter methylation, 21 Prostate s
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