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

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Table 1.10 Frequency of paediatric renal malignancies. Neoplasm Estimated relative frequency (%) Nephroblastoma 80 (nonanaplastic) Nephroblastoma 5 (anaplastic) Mesoblastic nephroma 5 Clear cell sarcoma 4 Rhabdoid tumour 2 Miscellaneous 4 Neuroblastoma Peripheral neuroectodermal tumour Synovial sarcoma Renal carcinoma Angiomyolipoma Lymphoma Other rare neoplasms WT1 allele {2043}. While WT1 alterations are strongly linked to the development of nephroblastoma in syndromic cases, their role in sporadic nephroblastoma is limited, with only one third of all nephroblastomas showing deletion at this locus and only 10% harbouring WT1 mutations. Beckwith-Wiedemann syndrome (characterized by hemihypertrophy, macroglossia, omphalocele, and visceromegaly) has been localized to chromosome 11p15, and designated WT2 although a specific gene has not been identified {747,1493,2077}. Attempts to determine the precise genetic event at this locus has revealed the presence of a cluster of imprinted genes; whether or not a single gene is responsible for the increased risk for nephroblastoma remains unclear {577}. The preferential loss of the maternal allele at this locus in cases of sporadic nephroblastoma suggests that genomic imprinting is involved in the pathogenesis of some tumours {2000}. Additional genetic loci are associated with familial nephroblastoma in patients with normal WT1 and WT2 {967, 1140,1141,1142,2134}. Approximately 1 percent of patients with nephroblastoma have a positive family history for the same neoplasm. Most pedigrees suggest autosomal dominant transmission with variable penetrance and expressivity. Prognosis and predictive factors Most nephroblastomas are of low stage, have a favourable histology, and are associated with an excellent prognosis. A favourable outcome can be expected even among most neoplasms with small foci of anaplasia. The most significant unfavourable factors are high stage, and the presence of anaplasia. The majority of blastemal tumours are exquisitely sensitive to therapy. However, tumours that demonstrate extensive blastemal cells following therapy are associated with poor response to therapy and reduced survival {197, 259}. In SIOP protocols, these blastemal chemoresistant tumours are classified as "high risk" and are treated like anaplastic tumours. 52 Tumours of the kidney
Nephrogenic rests and nephroblastomatosis E.J. Perlman L. Boccon-Gibod Definition Nephrogenic rests are abnormally persistent foci of embryonal cells that are capable of developing into nephroblastomas. Nephroblastomatosis is defined as the presence of diffuse or multifocal nephrogenic rests. Nephrogenic rests are classified into perilobar (PLNR) and intralobar (ILNR) types. Epidemiology Nephrogenic rests are encountered in 25% to 40% of patients with nephroblastoma, and in 1% of infant autopsies {190,192, 195,210,303}. Table 1.11 Features distinguishing perilobar from intralobar rests. Perilobar rests Intralobar rests Position in lobe Peripheral Random Margins Sharp, demarcated Irregular, intermingling Composition Blastema, tubules Stroma, blastema, tubules Stroma scant or sclerotic Stroma often predominates Distribution Usually multifocal Often unifocal Histopathology PLNRs and ILNRs have a number of distinguishing structural features. Perilobar nephrogenic rests PLNRs are sharply circumscribed and located at the periphery of the renal lobe. A PLNR may be dormant or may have several other fates: most commonly the rest will regress with peritubular scarring resulting in an obsolescent rest. PLNR may also undergo active proliferative overgrowth, resulting in hyperplastic nephrogenic rests, which can be almost impossible to distinguish from nephroblastoma. Rarely, PLNRs form a band around the surface of the kidney resulting in massive renal enlargement, (diffuse hyperplastic perilobar nephroblastomatosis). Nephroblastoma developing within a PLNR is recognized by its propensity for spherical expansile growth and a peritumoural fibrous pseudocapsule separating Fig. 1.72 Diffuse hyperplastic perilobar nephroblastomatosis (upper pole) with two spherical nephroblastomas and an separate perilobar nephrogenic rest in lower pole. Fig. 1.73 Perilobar nephrogenic rest. Note well demarcated, lens shaped subcapsular collection of blastemal and tubular cells. Nephroblastoma / Nephrogenic rests and nephroblastomatosis 53
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 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: increases in prevalence to approxim
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 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
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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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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
Page 309 and 310:
502. Corti B, Carella R, Gabusi E,
Page 311 and 312:
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
Page 353 and 354:
Promoter methylation, 21 Prostate s
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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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