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

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Chromophobe renal cell carcinoma S. Störkel G. Martignoni E. van den Berg Definition Renal carcinoma characterized by large pale cells with prominent cell membranes. ICD-O code 8317/3 Epidemiology Chromophobe renal cell carcinoma (CRCC) accounts for approximately 5 per cent of surgically removed renal epithelial tumours. The mean age of incidence is in the sixth decade, with a range in age of 27-86 years, and the number of men and women is roughly equal. Mortality is less than 10% {512}. Sporadic and hereditary forms exist. Clinical features There are no specific signs and symptoms. On imaging, these are mostly large masses without necrosis or calcifications. Macroscopy Chromophobe renal cell carcinomas are solid circumscribed tumours with slightly lobulated surfaces. In unfixed specimens the cut surface is homogeneously light Fig. 1.30 Chromophobe renal cell carcinoma (RCC). Typical homogeneously tan coloured tumour of the lower pole of the kidney. A B Fig. 1.31 Chromophobe RCC. A Chromophobe cells are arranged along vascular channels. B Note chromophobe and eosinophilic cells. A B Fig. 1.32 A Chromophobe RCC, eosinophilic variant. Note binucleated cells, perinuclear halos and tight intercellular cohesion. B Chromophobe RCC. Note typical granular cytoplasm with perinuclear clearance. 30 Tumours of the kidney
Fig. 1.33 Chromophobe RCC with sarcomatoid dedifferentiation. A Fig. 1.34 Chromophobe RCC. A Hale’s iron staining of eosinophilic variant. B Classic variant. Hale’s colloidal iron stain positivity in the cytoplasm. B brown or tan turning light grey after formalin fixation. Tumour spread and staging The majority of CRCCs are stage T1 and T2 (86%) whereas only 10% show extension through the renal capsule into surrounding adipose tissue, only 4% show involvement of the renal vein (T3b) {512}. A few cases of lymph node and distant metastasis (lung, liver and pancreas) have been described {152,1635,2172}. Histopathology In general, the growth pattern is solid, sometimes glandular, with focal calcifications and broad fibrotic septa. In contrast to clear cell renal cell carcinoma, many of the blood vessels are thick-walled and eccentrically hyalinized. The perivascular cells are often enlarged. Chromophobe renal cell carcinoma is characterized by large polygonal cells with transparent slightly reticulated cytoplasm with prominent cell membranes. These cells are commonly mixed with smaller cells with granular eosinophilic cytoplasm. The eosinophilic variant of chromophobe carcinoma is purely composed of intensively eosinophilic cells with prominent cell membranes {2610}. The cells have irregular, often wrinkled, nuclei. Some are binucleated. Nucleoli are usually small. Perinuclear halos are common. Sarcomatoid transformation occurs {2047}. Another diagnostic hallmark is a diffuse cytoplasmic staining reaction with Hale’s colloidal iron stain {475,2608}. Immunoprofile Immunohistology presents the following antigen profile: pan-Cytokeratin+, vimentin-, EMA+ (diffuse), lectins+, parvalbumin+, RCC antigen-/+, CD10– {140,1635,1675,2513}. Ultrastructure Electron microscopically, the cytoplasm is crowded by loose glycogen deposits and numerous sometimes invaginated vesicles, 150-300 nm in diameter resembling those of the intercalated cells type b of the cortical collecting duct {722,2515}. Somatic genetics Chromophobe renal cell carcinomas are characterized by extensive chromosomal loss, most frequently -1,-2,-6,-10,-13,-17 and –21 {338,2464}. The massive chromosomal losses lead to a hypodiploid DNA index {42}. Endoreduplication/polyploidization of the hypodiploid cells has been observed. Telomeric associations and telomere shortening have also been observed {1113,1375}. At the molecular level, Contractor et al. {486} showed that there are mutations of Fig. 1.35 Chromophobe RCC with typical monosomy (one signal for chromosome 17). FISH. Fig. 1.36 Chromophobe renal cell carcinoma. A representative karyotype of a chromophobe RCC showing extensive loss of chromosomes. Chromophobe renal cell carcinoma 31
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 and 24: Fig. 1.20 Clear cell renal cell car
Page 25 and 26: Papillary renal cell carcinoma B. D
Page 27: A B Fig. 1.29 Papillary renal cell
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
Page 75 and 76: Mixed epithelial and stromal tumour
Page 77 and 78: 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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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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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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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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