World Health Organization Classification of Tumours Pathology and ...

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Mucinous tubular and spindle cellcarcinomaJ.R. SrigleyDefinitionLow-grade polymorphic renal epithelialneoplasms with mucinous tubular andspindle cell features.EpidemiologyThere is a wide age range of 17-82(mean 53) years and a male to femaleratio of 1:4 {2024,2469}.Clinical featuresThey usually present as asymptomaticmasses, often found on ultrasound.Occasionally, they may present with flankpain or hematuria.MacroscopyMacroscopically, mucinous tubular andspindle cell carcinomas, are well circumscribedand have grey or light tan, uniformcut surfaces.villin are generally absent. Thesetumours show extensive positivity forUlex europaeus, peanut and soya beanagglutinins.UltrastructureThe spindle cells show epithelial featureslike tight junctions, desmosomes,microvillous borders, luminal bordersand occasional tonofilaments {2469}.Somatic geneticsUsing comparative genomic hybridizationand FISH, there is a characteristiccombination of chromosome losses, generallyinvolving chromosome 1, 4, 6, 8, 13and 14 and gains of chromosome 7, 11,16 and 17 {2137,2469}.Prognosis and predictive factorsThe prognosis sems to be favourable;only one example has been reported withmetastasis and this tumour is best consideredas a low-grade carcinoma{2471}.HistopathologyHistologically, they are composed oftightly packed, small, elongated tubulesseparated by pale mucinous stroma. Theparallel tubular arrays often have a spindlecell configuration sometimes simulatingleiomyoma or sarcoma. Many ofthese tumours had been previously diagnosedas unclassified or spindle cell(sarcomatoid) carcinomas.Individual cells are small with cuboidal oroval shapes and low-grade nuclear features.Occasionally, areas of necrosis,foam cell deposits and chronic inflammationmay be present. The mucinous stromais highlighted with stains for acidmucins.AImmunoprofileThese tumours have a compleximmunophenotype and stain for a widevariety of cytokeratins including lowmolecular weight keratins (CAM 5.2,MAK 6), CK7, CK18, CK19 and 34βE12{2469}. Epithelial membrane antigen iscommonly present, and vimentin andCD15 staining may be seen. Markers ofproximal nephron such as CD10 andBFig. 1.52 A, B, C Mucinous tubular and spindle cell carconoma composed of spindle cells and cuboidal cellsforming cords and tubules. Note basophilic extracellular mucin.C40 Tumours of the kidney
Papillary adenoma of the kidneyJ.N. EbleH. MochDefinitionPapillary adenomas are tumours with papillaryor tubular architecture of low nucleargrade and 5 mm in diameter or smaller.ICD-O code 8260/0Clinical featuresPapillary adenomas are the most commonneoplasms of the epithelium of therenal tubules. Autopsy studies havefound papillary adenomas increase infrequency in adulthood from 10% ofpatients younger than 40 years to 40% inpatients older than 70 years {653,2163,2854}. Similar lesions frequently develop inpatients on long-term hemodialysis andoccur in 33% of patients with acquired renalcystic disease {1143}.MacroscopyPapillary adenomas are well circumscribed,yellow to greyish white nodulesas small as less than 1 mm in diameter inthe renal cortex. Most occur just belowthe renal capsule. The smallest ones usuallyare spherical, but larger ones sometimesare roughly conical with a wedgeshapedappearance in sections cut atright angles to the cortical surface.Usually, papillary adenomas are solitary,but occasionally they are multiple and bilateral.When they are very numerous, thishas been called "renal adenomatosis".HistopathologyPapillary adenomas have tubular, papillary,or tubulopapillary architectures correspondingclosely to types 1 and 2 papillaryrenal cell carcinoma {585}. Somehave thin fibrous pseudocapsules. Thecells have round to oval nuclei with stippledto clumped chromatin and inconspicuousnucleoli; nuclear grooves maybe present. Mitotic figures usually areabsent. In most, the cytoplasm is scantand pale, amphophilic to basophilic.Less frequently, the cytoplasm is voluminousand eosinophilic, resembling type 2papillary renal cell carcinoma.Psammoma bodies are common, as arefoamy macrophages {2161}.Somatic geneticsLoss of the Y chromosome and a combinedtrisomy of chromosome 7 and 17are the first visible karyotype aberrationsin papillary renal tumours. This combinationof genetic alterations has been foundas the sole karyotype change in smallpapillary renal tumours from 2 mm to 5mm in diameter, all with nuclear grade 1{1373}. Based on these findings, it hasbeen suggested that papillary adenomasFig. 1.53 Multiple renal papillary adenomas.aquire additional genetic alterations duringgrowth, which change their biologicalbehaviour {1369}. One CGH analysis studied6 papillary tumours less than 6 mmin diameter and observed gain of chromosome7 in 4 specimens {2107}. Thesedata suggest that initiating genetic eventsfor papillary renal adenomas includegains of chromosome 7 and loss of a sexchromosome. Small renal tumours demonstratesimilar, but less extensive geneticalterations than their papillary renalcarcinoma counterparts. The clinicallyindolent course of small papillary tumoursmay, in part, be a result of the lower numberof genetic alterations per tumour.However, it is not possible to distinguishadenomas and carcinomas by geneticchanges, because many carcinomasshow only few genetic alterations.ABFig. 1.54 Papillary adenoma. A Two papillary adenomas in the renal cortex. These type 1 adenomas have complex papillae covered by a single layer of small epithelialcells with inconspicuous cytoplasm. B Papillary adenoma composed of complex branching papillae on partially hyalinized stromal cores.Mucinous tubular and spindle cell carcinoma / Papillary adenoma of the kidney41
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 and 28: ABFig. 1.29 Papillary renal cell ca
Page 29 and 30: Fig. 1.33 Chromophobe RCC with sarc
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: Renal cell carcinoma associated wit
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.
Page 79 and 80: Renal carcinoid tumourL.R. BéginDe
Page 81 and 82: Primitive neuroectodermal tumour(Ew
Page 83 and 84: Paraganglioma / PhaeochromocytomaPh
Page 85 and 86: LeukaemiaA. OraziInterstitial infil
Page 87 and 88: WHO histological classification of
Page 89 and 90:
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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