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

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Epithelioid angiomyolipoma M.B. Amin Definition Epithelioid angiomyolipoma (AML) is a potentially malignant mesenchymal neoplasm characterized by proliferation of predominantly epithelioid cells and is closely related to the triphasic (classic) AML. Epidemiology More than half of patients with epithelioid AML have a history of tuberous sclerosis (TS), which is a significantly higher association than classic AML has with TS {50,2036,1346}. Both sexes are equally affected similar to classic AML occurring in TS patients. The mean age of diagnosis is 38 years {649,50,463,466,593,1606,1634}. Clinical features Patients are frequently symptomatic, presenting with pain; some patients are discovered during TS follow-up. Imaging studies closely mimic renal cell carcinoma because of the paucity of adipose tissue {1289,463,224}. Macroscopy Tumours are usually large, with infiltrative growth and a grey-tan, white, brown or haemorrhagic appearance. Necrosis may be present. Extrarenal extension or involvement of the renal vein/vena cava may occur. Histopathology There is a proliferation of epithelioid cells with abundant granular cytoplasm arranged in sheets, often with perivascular cuffing of epithelioid cells. Many of the reported cases were initially misdiagnosed as a high grade carcinoma. Tumour cells are round to polygonal with enlarged vesicular nuclei often with prominent nucleoli. A B Fig. 1.100 Epithelioid angiomyolipoma. A Epithelioid angiomyolipoma is typically composed of a mixture of polygonal and spindle cells of variable size. Inflammatory cells often are mingled with the neoplastic cells. B Focally ganglion like and multinucleated cells are present. A B Fig. 1.101 Epithelioid angiomyolipoma. A Marked nuclear atypia and mitotic figures may be present. B Immunohistochemical reaction with HMB-45 shows numerous positive cells. 68 Tumours of the kidney
Multinucleated and enlarged ganglion-like cells may be present. A population of short spindle cells is present in many tumours. Tumours may display nuclear anaplasia, mitotic activity, vascular invasion, necrosis and infiltration of perinephric fat. Haemorrhage often is prominent. A few cases have focal classic AML areas {649,466}. Variations in histology include variable admixture of clear cells, although occasionally they may predominate {2184,560}. Immunoprofile Epithelioid AML expresses melanocytic markers (HMB-45, HMB-50, Mart- 1/Melan-A and microphthalmia transcription factor) with variable expression of smooth muscle markers (smooth muscle actin, muscle-specific actin) {125,1419, 2922,2511}. Genetics Allelic loss of chromosomal arm 16p (TS2 containing region) is noted in classic, epithelioid and sarcomatoid areas indicating clonality and relationship {2497}. TP53 mutation is detected in epithelioid but not triphasic AML, suggesting a role in malignant transformation {1289}. Prognostic and predictive factors Approximately one-third of epithelioid AML have been reported to have metastasis to lymph nodes, liver, lungs or spine {1565,1636,757,2863}. Among adverse pathologic parameters, none correlate with outcome; however, tumours with necrosis, mitotic activity, nuclear anaplasia and extrarenal spread should raise significant concern for malignant outcome {463,466,2036,757,2863}. Epithelioid angiomyolipoma 69
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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.
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 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: melanocytic and smooth muscle marke
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
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
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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
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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
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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
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Promoter methylation, 21 Prostate s
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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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