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

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makes 4.6% of lower urinary tract tumours and 0.07% of all cancers in Europe, and 5.2% and 0.07% respectively in North America. The highest incidence rates of renal pelvis tumours are observed in Australia, North America and Europe, while the lowest rates are noted in South and Central America and in Africa. The highest rates in males in 1990s were observed in Denmark (1.65/105), Ferrara Province in Italy (1.45/105), Hiroshima, Japan (1.41/105), and in Mallorca, Spain (1.38/105). In females, the highest incidence rates were noted in New South Wales and Queensland in Australia (1.34 and 1.03/105 respectively), Denmark (0.95/105), Louisiana (among Blacks), USA (0.79/105), and Iceland (0.79/105) {2016}. Although limited information is available about changes of renal pelvis cancer in time, available data from US show that in 1970s and 1980s renal pelvis cancer incidence rates rose by approximately 2.2% per year in both males and females {602}. Etiology of urothelial renal pelvis cancer Tobacco smoking Similar to cancers of the urinary bladder, the main risk factor for renal pelvis tumours is tobacco smoking {1680}. The relationship between tobacco smoking and renal pelvis tumours was reported already in 1970s {2324}, and confirmed by several authors {1215,1681,2245}. The risk increases with increasing lifetime consumption, as well as with Fig. 2.91 Ureter urothelial carcinoma. increasing intensity of smoking, and is similar in both sexes {1215,1681}. Analgetics Another proven risk factor for cancer of the renal pelvis is long-term use of analgesics, particularly phenacetin. Use of analgesics increases risk of renal pelvis tumours by 4-8 times in males and 10-13 times in women, even after elimination of the confounding effect of tobacco smoking {1668,1680,2245}. Occupational exposure Several occupations and occupational Fig. 2.92 Tumours of the ureter and renal pelvis. Inverted papillary urothelial carcinoma of the ureter with mutator phenotype. exposures have been reported to be associated with increased risk of renal pelvis tumours {1215}. The highest risk was found for workers of chemical, petrochemical and plastic industries, and also exposed to coke and coal, as well as to asphalt and tar {1215}. Other risk factors include papillary necrosis, Balkan nephropathy, thorium containing radiologic contrast material, urinary tract infections or stones {922, 1227,1260,1583}. Macroscopy Tumours may be papillary, polypoid, A B Fig. 2.93 Tumours of the ureter and renal pelvis. A Partly papillary predominamtly inverted growth pattern (a,c) with cytological atypia. B Inverted papillary urothelial carcinoma of the ureter with mutator phenotype. Tumours of the renal pelvis and ureter 151
nodular, ulcerative or infiltrative. Some tumours distend the entire pelvis while others ulcerate and infiltrate, causing thickening of the wall. A high grade tumour may appear as an ill defined scirrhous mass that involves the renal parenchyma, mimicking a primary renal epithelial neoplasm. Hydronephrosis and stones may be present in renal pelvic tumours while hydroureter and/or stricture may accompany ureteral neoplasms. Multifocality must be assessed in all nephroureterectomy specimens. Tumour staging There is a separate TNM staging system for tumours of the renal pelvis and ureter {944,2662}. Slight differences based on anatomical distinctions exist in the pT 3 designation of renal pelvis and ureteral tumours. Histopathology The basic histopathology of renal pelvis urothelial malignancies mirrors bladder urothelial neoplasia and may occur as papillary non-invasive tumours (papillary urothelial neoplasm of low malignant potential, low grade papillary carcinoma or high grade papillary carcinoma), carcinoma-in-situ and invasive carcinoma. The entire morphologic spectrum of vesical urothelial carcinoma is seen and tumour types include those showing aberrant differentiation (squamous and glandular), unusual morphology (nested, microcystic, micropapillary, clear cell and plasmacytoid) and poorly differentiated carcinoma (lymphoepithelioma-like, sarcomatoid and giant cell) {355,399, 656,727,2706}. Concurrence of aberrant differentiation, unusual morphology or undifferentiated carcinoma with conventional invasive poorly differentiated carcinoma is frequent. Grading The grading system for urothelial tumours is identical to that employed for bladder tumours. Fig. 2.94 Loss of expression of the DNA mismatch repair gene MLH1 in an area of low grade urothelial dysplasia. Fig. 2.95 Tumours of the ureter and renal pelvis. Microsatellite instability in 4 markers of the consensus Bethesda panel {264}. Genetics Urothelial carcinomas of the renal pelvis, ureter and urinary bladder share similar genetic alterations {734,2197}. Deletions on chromosome 9p and 9q occur in 50- 75% of all patients {734,993,2197,2554} and frequent deletions at 17p in addition to p53 mutations, are seen in advanced invasive tumours {321,993}. 20-30% of all upper urinary tract cancers demonstrate microsatellite instability and loss of the mismatch repair proteins MSH2, MLH1 or MSH6 {251,1032,1507}. Mutations in genes with repetitive sequences in the coding region (TGFβRII, bax, MSH3, MSH6) are found in 20-33% of cases with MSI, indicating a molecular pathway of carcinogenesis that is similar to some mismatch repair-deficient colorectal cancers. Tumours with microsatellite instability have significantly different clinical and histopathological features including low tumour stage and grade, papillary and frequently inverted growth pattern and a higher prevalence in female patients {1028,1032}. Prognosis and predictive factors The most important prognostic factor is tumour stage and for invasive tumours the depth of invasion. A potential pitfall is that, while involvement of the renal parenchyma is categorized as a pT 3 tumour, some tumours that invade the muscularis (pT 2 ) may show extension into renal tubules in a pagetoid or intramucosal pattern and this should not be designated as pT 3 . Survival for patients with pT a /pT is lesions is essentially 100%, Genetic susceptibility Familial history of kidney cancer {2245} is generall considered a risk factor. Urothelial carcinomas of the upper urothelial tract occur in the setting of hereditary nonpolyposis colorectal cancer (HNPCC) syndrome (Lynch syndrome II) {251}. Fig. 2.96 Lymphoepithelioma-like urothelial carcinoma of the ureter. Inset shows cytokeratin AE1/AE3 immunostain. 152 Tumours of the urinary system
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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
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peritumoural fibrous pseudocapsule.
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increases in prevalence to approxim
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Nephrogenic rests and nephroblastom
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Cystic partially differentiated nep
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A B Fig. 1.80 Clear cell sarcoma of
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A B Fig. 1.85 Rhabdoid tumour of th
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transcription factor is fused to th
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Leiomyosarcoma S.M. Bonsib Definiti
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Angiomyolipoma G. Martignoni M.B. A
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melanocytic and smooth muscle marke
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Multinucleated and enlarged ganglio
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Haemangioma P. Tamboli Definition H
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Fig. 1.107 Juxtaglomerular cell tum
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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
Page 97 and 98: A Fig. 2.12 Infiltrative urothelial
Page 99 and 100: A Fig. 2.15 A Infiltrating urotheli
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: Tumours of the renal pelvis and ure
Page 151 and 152: Tumours of the urethra F. Hofstädt
Page 153 and 154: usually show enteric, colloid or si
Page 155 and 156: CHAPTER 3X Tumours of of the the Pr
Page 157 and 158: TNM classification of carcinomas of
Page 159 and 160: contrast, mortality among migrants
Page 161 and 162: Fig. 3.06 Transrectal ultrasound of
Page 163 and 164: PSA-related diagnostic strategies.
Page 165 and 166: A B C Fig. 3.10 A,B Section of pros
Page 167 and 168: pale-clear, similar to benign gland
Page 169 and 170: A B Fig. 3.20 A, B Adenocarcinoma w
Page 171 and 172: prostate adenocarcinomas exhibit AR
Page 173 and 174: A Fig. 3.27 A, B Foamy gland adenoc
Page 175 and 176: A B Fig. 3.32 A Sarcomatoid carcino
Page 177 and 178: A B Fig. 3.37 A Gleason score 1+1=2
Page 179 and 180: A B Fig. 3.43 A Prostate cancer Gle
Page 181 and 182: Fig. 3.48 Heat map-nature. From S.M
Page 183 and 184: Fig. 3.51 Prostate cancer. Major su
Page 185 and 186: many stage T1b cancers. Stage T2 Mo
Page 187 and 188: Fig. 3.58 Patterns of seminal vesic
Page 189 and 190: Prostatic intraepithelial neoplasia
Page 191 and 192: A B Fig. 3.63 A Micropapillary high
Page 193 and 194: A B Fig. 3.68 A Ductal carcinoma in
Page 195 and 196: Ductal adenocarcinoma X.J. Yang L.
Page 197 and 198: 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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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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