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

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1553}. The etiopathogenesis of this tumour is unknown, although it is suspected that it originates from modified urothelial cells, that are possibly derived from basal (stem) cells {1106}. Hybridization with <strong>Epstein</strong>-Barr virus encoded RNA has been reported to be consistently negative in different series {82,973,1106,1553}. The tumour is solitary and usually involves the dome, posterior wall, or trigone, often with a sessile growth pattern. Lymphoepithelioma-like carcinoma may be pure, predominant or focally admixed with typical urothelial carcinoma, or in some cases with squamous cell carcinoma or adenocarcinoma {1106,1553}. The proportion of lymphoepithelioma-like carcinoma histology should be provided in tumours with mixed histology. Histologically, the tumour is composed of nests, sheets, and cords of undifferentiated cells with large pleomorphic nuclei and prominent nucleoli. The cytoplasmic borders are poorly defined imparting a syncytial appearance. The background consists of a prominent lymphoid stroma that includes T and B lymphocytes, plasma cells, histiocytes, and occasional neutrophils or eosinophils, the latter being prominent in rare cases. Carcinoma in situ elsewhere in the bladder is rarely present. The epithelial cells of this tumour stain with several cytokeratin (CK) markers as follows: AE1/AE3, CK8, CK 7, and they are rarely positive for CK20 {1106,1553}. In some cases, it is possible to overlook the malignant cells in the background of inflamed bladder wall and misdiagnose the condition as florid chronic cystitis {1553}. The major differential diagnostic considerations are poorly differentiated urothelial carcinoma with lymphoid stroma; poorly differentiated squamous cell carcinoma, and lymphoma {1553}. The presence of recognizable urothelial or squamous cell carcinoma does not exclude lymphoepithelioma-like carcinoma; rather, the diagnosis is based on finding areas typical of lymphoepithelioma-like carcinoma reminiscent of that in the nasopharynx. Differentiation from lymphoma may be difficult, but the presence of a syncytial pattern of large malignant cells with a dense polymorphous lymphoid background is an important clue {1553}. Most reported cases of the urinary bladder had a relatively favourable prognosis A B Fig. 2.14 Lymphoepithelioma-like carcinoma of the urinary bladder. A Characteristic syncytial appearance of neoplastic cells (H&E). B Note the characteristic immunostaining with CK. when pure or predominant, but when lymphoepithelioma-like carcinoma is focally present in an otherwise typical urothelial carcinoma, these patients behave like patients with conventional urothelial carcinoma alone of the same grade and stage {1106,1553}. Some examples of lymphoepithelioma-like carcinoma have been described in the ureter and the renal pelvis {820,2224}. This tumour, thus far has been found to be responsive to chemotherapy when it is encountered in its pure form {82,623}. Experience at one institution has shown a complete response to chemotherapy and transurethral resection of the bladder {82,623}. Another series of nine patients treated with a combination of transurethral resection, partial or complete cystectomy, and radiotherapy disclosed four patients without evidence of disease, three who died of their disease and two who died of other causes {1106}. Lymphoma-like and plasmacytoid variants The lymphoma-like and plasmacytoid variants of urothelial carcinoma are those in which the malignant cells resemble those of malignant lymphoma or plasmacytoma {1618,2272,2571,2933,2949}. Less than 10 cases have been reported. The histologic features of the lymphomalike and plasmacytoid variants of urothelial carcinoma are characterized by the presence of single malignant cells in a loose or myxoid stroma. The tumour cells have clear or eosinophilic cytoplasm and eccentrically placed, enlarged hyperchromatic nuclei with small nucleoli. Almost all of the reported cases have had a component of high grade urothelial carcinoma in addition to the single malignant cells. In some of the cases, the single-cell component was predominant on the initial biopsy, leading to the differential diagnosis of lymphoma/plasmacytoma. The tumour cells stain with cytokeratin (CK) cocktail, CK 7 and (in some cases) CK 20 {2571}. Immunohistochemical stains for lymphoid markers have consistently been reported as negative. Each of these variants of urothelial carcinoma may cause a significant differential diagnostic dilemma, especially in cases in which it constitutes the predominant or Infiltrating urothelial carcinoma 101
A Fig. 2.15 A Infiltrating urothelial carcinoma of the bladder, plasmocytoid variant. B Plasmacytoid variant of urothelial carcinoma of the urinary bladder. exclusive component in a small biopsy sample. The importance of recognizing these variants lies in not mistaking them as a lymphoma or plasmacytoma. Limited information is available about the outcome of patients with these variants of urothelial carcinoma. Of 6 cases reported by Tamboli et al. {2571} 4 died of their disease, one died post-operatively and one is alive without evidence of disease. Sarcomatoid variant (with/without heterologous elements) The term sarcomatoid variant of urothelial carcinoma should be used for all biphasic malignant neoplasms exhibiting morphologic and/or immunohistochemical evidence of epithelial and mesenchymal differentiation (with the presence or absence of heterologous elements acknowledged in the diagnosis). There is considerable confusion and disagreement in the literature regarding nomenclature and histogenesis of these tumours. In some series, both carcinosarcoma and sarcomatoid carcinoma are included as "sarcomatoid carcinoma" {2175}. In others they are regarded as separate entities. The mean age is 66 years (range, 50-77 years old) and most patients present with hematuria {1555,2175}. A previous history of carcinoma treated by radiation or the exposition to cyclophosphamide therapy is common {1551}. Rare examples of carcinosarcoma and sarcomatoid carcinomas have been described in the ureter and the renal pelvis {1549}. The gross appearance is characteristically "sarcoma-like", dull grey with infiltrative margins. The tumours are often polypoid with large intraluminal masses. Microscopically, sarcomatoid carcinoma is composed of urothelial, glandular or small cell component showing variable degrees of differentiation {1555}. A small B A C subset of sarcomatoid carcinoma may have a prominent myxoid stroma {1238}. The mesenchymal component most frequently observed is an undifferentiated high grade spindle cell neoplasm. The most common heterologous element is osteosarcoma followed by chondrosarcoma, rhabdomyosarcoma, leiomyosarcoma, liposarcoma angiosarcoma or multiple types of heterologous differentiation may be present {957,1238,1549, 1555,2175}. By immunohistochemistry, epithelial elements react with cytokeratins, whereas stromal elements react with vimentin or specific markers corresponding to the mesenchymal differentiation. The sarcomatoid phenotype retains the epithelial nature of the cells by immunohistochemistry or electronmicroscopy {1549,1555}. Recent molecular studies, strongly argue for a monoclonal origin of both components {957}. The cytological atypia of sarcomatoid carcinoma excludes non-neoplastic lesions such as the postoperative spindle cell nodule and inflammatory pseudotumour {1161,1550}. Sarcomatoid carcinoma should be distinguished from the rare carcinoma with metaplastic, benign-appearing bone or cartilage in the stroma or those showing other pseudosarcomatous stromal reactions. Nodal and distant organ metastases at diagnosis are common {957,1555,1960, 2175} and 70% of patients died of cancer at 1 to 48 months (mean 17 months) {1555} Urothelial carcinoma with giant cells High grade urothelial carcinoma may contain epithelial tumour giant cells or the tumour may appear undifferentiated resembling giant cell carcinoma of the lung. This variant is very infrequent. It must be distinguished from occasional cases showing giant cells (osteoclastic or foreign body type) in the stroma or urothelial carcinoma showing trophoblastic differentiation. In some cases the giant cell reaction is so extensive that it may mimic giant cell tumour of the bone {2948}. Fig. 2.16 A Infiltrative urothelial carcinoma. Sarcomatoid variant without heterologous elements showing spindle cell morphology. B Infiltrating urothelial carcinoma of the bladder. Sarcomatoid variant with heterologous smooth muscle elements. C Immunohistochemical expression of cytokeratin AE1/AE3 in a case of sarcomatoid carcinoma of the urinary bladder (same case as in panel A). D Immunohistochemical expression of smooth muscle actin of the sarcomatoid carcinoma shown in panel B. B D 102 Tumours of the urinary system
Page 1 and 2:
World Health Organization Classific
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This volume was produced in collabo
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Contents 1 Tumours of the kidney 9
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CHAPTER 1 Tumours of the Kidney Can
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TNM classification of renal cell ca
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one quarter of kidney cancers in bo
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Familial renal cell carcinoma M.J.
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Table 1.02 Genotype - phenotype cor
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A B Fig. 1.11 A Multiple cutaneous
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A B Fig. 1.14 Birt-Hogg-Dubé syndr
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Clear cell renal cell carcinoma D.J
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Fig. 1.20 Clear cell renal cell car
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Papillary renal cell carcinoma B. D
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A B Fig. 1.29 Papillary renal cell
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Fig. 1.33 Chromophobe RCC with sarc
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Carcinoma of the collecting ducts o
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Renal medullary carcinoma C.J. Davi
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Renal carcinomas associated with Xp
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Renal cell carcinoma associated wit
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Papillary adenoma of the kidney J.N
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of this tumour. Microscopic extensi
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A B Fig. 1.59 Metanephric adenoma.
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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.
Page 79 and 80: Renal carcinoid tumour L.R. Bégin
Page 81 and 82: Primitive neuroectodermal tumour (E
Page 83 and 84: Paraganglioma / Phaeochromocytoma P
Page 85 and 86: Leukaemia A. Orazi Interstitial inf
Page 87 and 88: WHO histological classification of
Page 89 and 90: TNM classification of carcinomas of
Page 91 and 92: The risk of bladder cancer goes dow
Page 93 and 94: Tumour spread and staging Urinary b
Page 95 and 96: feature in such patients undergoing
Page 97: A Fig. 2.12 Infiltrative urothelial
Page 101 and 102: A B Fig. 2.19 A Infiltrative urothe
Page 103 and 104: Fig. 2.23 Infiltrative urothelial c
Page 105 and 106: ing systems have been proposed on t
Page 107 and 108: Non-invasive urothelial tumours G.
Page 109 and 110: chronically inflamed urothelium and
Page 111 and 112: Inverted papilloma G. Sauter Defini
Page 113 and 114: muscle invasive disease, but there
Page 115 and 116: Fig. 2.42 Non-invasive urothelial n
Page 117 and 118: A Fig. 2.45 Non-invasive urothelial
Page 119 and 120: for DBCCR1 silencing {984,2476}. Th
Page 121 and 122: Squamous cell carcinoma D.J. Grigno
Page 123 and 124: Fig. 2.51 Squamous cell carcinoma.
Page 125 and 126: Adenocarcinoma A.G. Ayala P. Tambol
Page 127 and 128: A B Fig. 2.61 A Adenocarcinoma in s
Page 129 and 130: A B Fig. 2.65 Intramural urachal ca
Page 131 and 132: Müllerian origin is postulated for
Page 133 and 134: A Fig. 2.69 Small cell carcinoma. A
Page 135 and 136: Carcinoid L. Cheng Definition Carci
Page 137 and 138: Leiomyosarcoma J. Cheville Definiti
Page 139 and 140: Osteosarcoma L. Guillou Definition
Page 141 and 142: Leiomyoma J. Cheville Definition A
Page 143 and 144: Haemangioma L. Cheng Definition Hae
Page 145 and 146: Metastatic tumours and secondary ex
Page 147 and 148: 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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WHO histological classification of
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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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WHO histological classification of
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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
Page 347 and 348:
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
Page 353 and 354:
Promoter methylation, 21 Prostate s
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