World Health Organization Classification of Tumours Pathology and ...

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Chromophobe renal cell carcinomaS. StörkelG. MartignoniE. van den BergDefinitionRenal carcinoma characterized by largepale cells with prominent cell membranes.ICD-O code 8317/3EpidemiologyChromophobe renal cell carcinoma(CRCC) accounts for approximately 5per cent of surgically removed renalepithelial tumours. The mean age of incidenceis in the sixth decade, with arange in age of 27-86 years, and thenumber of men and women is roughlyequal. Mortality is less than 10% {512}.Sporadic and hereditary forms exist.Clinical featuresThere are no specific signs and symptoms.On imaging, these are mostly largemasses without necrosis or calcifications.MacroscopyChromophobe renal cell carcinomas aresolid circumscribed tumours with slightlylobulated surfaces. In unfixed specimensthe cut surface is homogeneously lightFig. 1.30 Chromophobe renal cell carcinoma (RCC).Typical homogeneously tan coloured tumour of thelower pole of the kidney.ABFig. 1.31 Chromophobe RCC. A Chromophobe cells are arranged along vascular channels. B Note chromophobe and eosinophilic cells.ABFig. 1.32 A Chromophobe RCC, eosinophilic variant. Note binucleated cells, perinuclear halos and tight intercellular cohesion. B Chromophobe RCC. Note typicalgranular cytoplasm with perinuclear clearance.30 Tumours of the kidney
Fig. 1.33 Chromophobe RCC with sarcomatoid dedifferentiation.AFig. 1.34 Chromophobe RCC. A Hale’s iron staining of eosinophilic variant. B Classic variant. Hale’s colloidaliron stain positivity in the cytoplasm.Bbrown or tan turning light grey after formalinfixation.Tumour spread and stagingThe majority of CRCCs are stage T1 andT2 (86%) whereas only 10% show extensionthrough the renal capsule into surroundingadipose tissue, only 4% showinvolvement of the renal vein (T3b) {512}.A few cases of lymph node and distantmetastasis (lung, liver and pancreas)have been described {152,1635,2172}.HistopathologyIn general, the growth pattern is solid,sometimes glandular, with focal calcificationsand broad fibrotic septa. In contrastto clear cell renal cell carcinoma, manyof the blood vessels are thick-walled andeccentrically hyalinized. The perivascularcells are often enlarged.Chromophobe renal cell carcinoma ischaracterized by large polygonal cellswith transparent slightly reticulated cytoplasmwith prominent cell membranes.These cells are commonly mixed withsmaller cells with granular eosinophiliccytoplasm. The eosinophilic variant ofchromophobe carcinoma is purely composedof intensively eosinophilic cellswith prominent cell membranes {2610}.The cells have irregular, often wrinkled,nuclei. Some are binucleated. Nucleoliare usually small. Perinuclear halos arecommon. Sarcomatoid transformationoccurs {2047}. Another diagnostic hallmarkis a diffuse cytoplasmic stainingreaction with Hale’s colloidal iron stain{475,2608}.ImmunoprofileImmunohistology presents the followingantigen profile: pan-Cytokeratin+,vimentin-, EMA+ (diffuse), lectins+, parvalbumin+,RCC antigen-/+, CD10–{140,1635,1675,2513}.UltrastructureElectron microscopically, the cytoplasmis crowded by loose glycogen depositsand numerous sometimes invaginatedvesicles, 150-300 nm in diameter resemblingthose of the intercalated cells typeb of the cortical collecting duct{722,2515}.Somatic geneticsChromophobe renal cell carcinomas arecharacterized by extensive chromosomalloss, most frequently -1,-2,-6,-10,-13,-17and –21 {338,2464}.The massive chromosomal losses lead toa hypodiploid DNA index {42}.Endoreduplication/polyploidization of thehypodiploid cells has been observed.Telomeric associations and telomereshortening have also been observed{1113,1375}.At the molecular level, Contractor et al.{486} showed that there are mutations ofFig. 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 showingextensive loss of chromosomes.Chromophobe renal cell carcinoma31
Page 1 and 2: World Health Organization Classific
Page 3 and 4: This volume was produced in collabo
Page 5 and 6: Contents1 Tumours of the kidney 9WH
Page 7 and 8: CHAPTER 1Tumours of the KidneyCance
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 carcinomaM.J. M
Page 15 and 16: Table 1.02Genotype - phenotype corr
Page 17 and 18: ABFig. 1.11 A Multiple cutaneous le
Page 21 and 22: Clear cell renal cell carcinomaD.J.
Page 23 and 24: Fig. 1.20 Clear cell renal cell car
Page 25 and 26: Papillary renal cell carcinomaB. De
Page 27: ABFig. 1.29 Papillary renal cell ca
Page 31 and 32: Carcinoma of the collectingducts of
Page 33 and 34: Renal medullary carcinomaC.J. Davis
Page 35 and 36: Renal carcinomas associated withXp1
Page 37 and 38: Renal cell carcinoma associated wit
Page 39 and 40: Papillary adenoma of the kidneyJ.N.
Page 41 and 42: of this tumour. Microscopic extensi
Page 43 and 44: ABFig. 1.59 Metanephric adenoma. A
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 andnephroblastoma
Page 53 and 54: Cystic partially differentiatedneph
Page 55 and 56: ABFig. 1.80 Clear cell sarcoma of t
Page 57 and 58: ABFig. 1.85 Rhabdoid tumour of the
Page 59 and 60: transcription factor is fused to th
Page 61 and 62: LeiomyosarcomaS.M. BonsibDefinition
Page 63 and 64: AngiomyolipomaG. MartignoniM.B. Ami
Page 65 and 66: melanocytic and smooth muscle marke
Page 67 and 68: Multinucleated and enlarged ganglio
Page 69 and 70: HaemangiomaP. TamboliDefinitionHaem
Page 71 and 72: Fig. 1.107 Juxtaglomerular cell tum
Page 73 and 74: Intrarenal schwannomaI. Alvarado-Ca
Page 75 and 76: Mixed epithelial and stromal tumour
Page 77 and 78: Synovial sarcoma of the kidneyJ.Y.
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Renal carcinoid tumourL.R. BéginDe
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Primitive neuroectodermal tumour(Ew
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Paraganglioma / PhaeochromocytomaPh
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LeukaemiaA. OraziInterstitial infil
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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 stagingUrinary bl
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feature in such patients undergoing
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AFig. 2.12 Infiltrative urothelial
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AFig. 2.15 A Infiltrating urothelia
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it difficult to identify genes lead
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Alterations of 9p21 and p15/p16 bel
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nostic role in invasively growing b
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Urothelial hyperplasiaJ.I. EpsteinU
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Urothelial papillomaC. BuschS.L. Jo
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Somatic geneticsUltrastructure, ant
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In spite of the overall orderly app
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Urothelial carcinoma in situI.A. Se
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aberrations, often including high l
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grade carcinomas recur frequently (
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AFig. 2.47 Squamous cell carcinoma.
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grading to be a significant morphol
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AFig. 2.57 Adenocarcinoma. A High p
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Urachal carcinomaA.G. AyalaP. Tambo
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Clear cell adenocarcinomaE. OlivaDe
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Small cell carcinomaF. AlgabaG. Sau
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mon in females by 1.4:1 {1845}. The
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RhabdomyosarcomaI. LeuschnerDefinit
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AngiosarcomaJ. ChevilleDefinitionAn
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Malignant fibrous histiocytomaJ. Ch
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Granular cell tumourI.A. Sesterhenn
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LymphomasA. MarxDefinitionMalignant
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ABCFig. 2.87 Metastatic tumours to
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makes 4.6% of lower urinary tracttu
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and patients with pT 2 tumours have
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Table 2.07Anatomic classification o
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prominent basal epithelial cell lay
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WHO histological classification of
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Acinar adenocarcinomaJ.I. EpsteinF.
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have been seen in high-risk countri
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ABCFig. 3.07 A Pelvic metastases of
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expression of PSMA in serum of both
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AFig. 3.12 A Organ-confined adenoca
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glands. These are perineural invasi
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have occasional p63 immunoreactive
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ABFig. 3.25 A Pseudohyperplastic ad
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PSA positive. Associated acinar ade
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AAFig. 3.36 Schematic diagram of th
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AFig. 3.39 A Gleason score 3+3=6. B
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and prostatectomy Gleason score{375
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Table 3.01Prostate cancer susceptib
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AFig. 3.52 A Immunohistochemistry f
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ABFig. 3.57 A Pathological stage an
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Fig. 3.59 Preoperative PSA levels (
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ABFig. 3.61 A Flat and tufting patt
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ABFig. 3.65 High grade PIN with foa
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Table 3.04Risk of subsequent carcin
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sue. Transrectal needle core biopsi
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Urothelial carcinomaD.J. GrignonDef
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CK20 in the majority of cases and h
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Basal cell carcinomaP.H. TanA. Bill
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ABFig. 3.84 Small cell carcinoma. A
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ABFig. 3.87 A Malignant phyllodes t
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Haematolymphoid tumoursK.A. Iczkows
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gliomas should not be designated as
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CHAPTER 4XTumours of the of the Tes
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TNM classification of germ cell tum
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Germ cell tumoursP.J. WoodwardA. He
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senting symptom: back or abdominalp
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ABFig. 4.03 Germ cell tumours genet
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process at all {1524}. In addition,
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ABFig. 4.12 Comparison of morpholog
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small tumour insufficient to produc
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ABCFig. 4.22 Seminoma. A Pseudoglan
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ABFig. 4.27 Spermatocytic seminoma.
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Differential diagnosesDifferential
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Histologic patternsMicrocystic or r
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AFig. 4.40 Yolk sac tumour. A Pleom
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TeratomaTeratomas are generally wel
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ABCFig. 4.49 Teratoma. A Teratoma w
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ABFig. 4.52 Teratoma with somatic t
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ABFig. 4.59 Mixed germ cell tumours
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ABCFig. 4.64 Leydig cell tumour. A
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AFig. 4.69 Androgen insensitivity s
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tinguished from Sertoli cell nodule
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ound and have spherical, regularly
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Tumours containing both germ cell a
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Miscellaneous tumours of the testis
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Lymphoma and plasmacytoma of thetes
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Tumours of collecting ducts and ret
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Tumours of paratesticular structure
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EpidemiologyNodular mesothelial hyp
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ABFig. 4.104 Papillary cystadenoma
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Fig. 4.109 Desmoplastic small round
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Fig. 4.115 Leiomyosarcoma. Coronal,
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Secondary tumoursC.J. DavisDefiniti
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CHAPTER 5Tumours of the PenisThe in
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Malignant epithelial tumoursA.L. Cu
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ABFig. 5.05 A Well differentiated s
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ABFig. 5.10 Squamous cell carcinoma
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Fig. 5.17 Low grade papillary carci
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5 cm in diameter. The term has been
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Melanocytic lesionsA.G. AyalaP. Tam
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Fig. 5.29 Lobular capillary haemang
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ABFig. 5.34 A Kaposi sarcoma of the
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LymphomasA. MarxDefinitionPrimary p
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ContributorsDr Lauri A. AALTONENRes
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Dr Kyu Rae KIMDepartment of Patholo
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Dr Thomas M. ULBRIGHT*Department of
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3.03.01 Dr D.M. Parkin03.02 WHO/NCH
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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,C
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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, 226CCND1, 105, 108CCND2, 226,
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JJuvenile type granulosa cell tumou
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Promoter methylation, 21Prostate sp
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