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

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epresenting tumour cells undergoing pyknosis are prominent in some cases. Mitotic figures may be numerous. Though the nuclear to cytoplasmic ratio is high, a rim of clear cytoplasm and discrete cell membranes are often apparent in well-fixed tumours without extensive degenerative changes. The presence of clear cytoplasm is often associated with abundant glycogen as demonstrated by diastase sensitive PAS-positivity. Immunoprofile The basic immunophenotype of PNET- EWS, regardless of the primary site, is the expression of vimentin and the surface antigen of the MIC2 gene, CD99 (O13) or HBA-71. Approximately 20% of cases also express pan-cytokeratin. The staining pattern for vimentin and cytokeratin may be perinuclear or Golgi zone punctate reactivity. Somatic genetics Virtually all of the recently reported PNET- EWSs have had the t(11;22)(q24;q12) translocation with the fusion transcript between the EWS gene (22q12) and the ETS-related oncogene, FLI1 (11q24) {1627,2124}. Variant translocations with EWS are those with other ETS-related oncogenes: (21q22), (7p22), (17q12) and (2q33). Prognosis Pathologic stage is the major determinant in the prognosis of PNET-EWS regardless of the primary site. Aggressive multidrug chemotherapy has resulted in an improvement in the clinical outcome {525}. Fig. 1.128 PNET of the kidney. CD99 expression. Table 1.12 Immunohistochemical differentiation of neuroectodermal tumours from other tumours with similar microscopic features. VIM CK CHR SYN NSE CD99 CD45 WT-1 CD117 PNET-EWS + +/– +/– +/– + + – – + NB – – + + + – – – + Carcinoid – + + + + – – – – NEC – + + + + – – – – NHL + – – – – +* + – – Blastemal WT + + – – +/– – – + + ________ Abbreviations: PNET-EWS = primitive neuroectodermal tumour / Ewing sarcoma, NB= neuroblastoma, NEC= neuroendocrine carcinoma, NHL= non-Hodgkin lymphoma, WT = Wilms tumour, VIM = vimentin, CK = cytokeratin, CHR = chromogranin, SYN = synaptophysin, NSE - neuron specific enolase. * CD99 is expressed by lymphoblastic lymphoma. Neuroblastoma D.M. Parham ICD-O code 9500/3 Neuroblastomas arising as a true intrarenal mass are extremely rare; only six cases were identified in the National Wilms Tumour Study Pathology Centre in 1993 {2225}. Pure intrarenal lesions hypothetically arise from either adrenal rests or intrarenal sympathetic tissue {2385}. Far more frequently, adrenal neuroblastomas invade the adjacent kidney; this occurs in approximately five per cent of cases {2375}. Because most neuroblastomas arise from the adrenal, those affecting the kidney predominate in the superior pole. Extensive renal sinus invasion may simulate a pelvic tumour. Preoperative determination of urine catecholamine excretion is helpful in diagnosis of neuroblastoma but may not exclude nephroblastomas with neural elements {2273}. The presence of primitive neural tissue defines neuroblastomas, which contain Homer Wright rosettes, neurofibrillary stroma, and embryonal cells with round nuclei containing granular, "salt and pepper" chromatin. Important positive indicators of neuronal differentiation include neuronspecific enolase, synaptophysin, S100 protein, and chromogranin. 84 Tumours of the kidney
Paraganglioma / Phaeochromocytoma Ph.U. Heitz ICD-O codes Paraganglioma 8680/1 Pheochromocytoma 8700/0 A very small number of tumours have been described in the kidney {595,1426}. Most tumours are small. The cut surface is grey, often well vascularized. The colour of the parenchyma often rapidly turns brown when exposed to air. This is due to oxidation of chromaffin substances, including catecholamines. The architecture is characterized by cell clusters ("Zellballen") surrounded by a network of fine collagenous septa, containing blood vessels and sustentacular cells. The immunoreactions for synaptophysin, chromogranin A, and CD56 are consistently strong in virtually all tumour cells. Protein S-100 highlights tumour cells and sustentacular cells. Lymphomas A. Marx S.M. Bonsib Definition Primary renal lymphoma is a lymphoma without evidence of systemic involvement. Epidemiology Less than 100 cases of primary renal lymphomas, both Hodgkin disease and non-Hodgkin lymphoma, have been described. However, post-transplant lymphoproliferative disorders are the most frequently encountered disorder today. In the non-transplant patients, primary lymphomas may present as a mass lesion and regarded clinically as a renal epithelial neoplasm and treated by nephrectomy. The diagnosis requires renal and bone marrow biopsy and thoraco-abdominal CT {2477}. Dissemination following the diagnosis of PRL is common. Secondary renal lymphomas (SRL) affect the kidney as the second most common site for metastasis {2284}. It is 30x more common than PRL {374,537}. Most present (48%) in advanced stage lymphoma {1267}. Etiology PRL arising in transplanted kidneys are usually EBV-associated monomorphic or polymorphic B-cell lymphoproliferations of donor origin and related to iatrogenic immunosuppression {439,839,1695,2833}. Clinical features Common symptoms are flank or abdominal pain, haematuria, fever, weight loss, hypertension, renal insufficiency, or acute renal failure {448,537,626,1354, 2097,2382}. Complications are renal failure {750} and paraneoplastic hypercalcemia {2676}. Macroscopy Nephrectomy specimens in primary or secondary lymphoma show single or multifocal nodules (eventually associated with hydronephrosis) or diffuse renal enlargment. In secondary lymphoma, bilateral involvement is frequent (10% to 30%) {13,1881,2097,2408,2647,2696}. The cut surface is usually homogeneous, firm and pale, but necrosis, haemorrhage, cystic changes, calcifications and tumoral thrombus formation in the renal vein may occur {2677,2760}. Intravascular large B-cell lymphoma almost always affects the kidneys but may cause no macroscopic change {2819}. Histopathology There are three patterns of renal involvement. The most common is diffuse involvement with lymphoma cells permeating between the native nephron structures resulting in marked organ enlargement. The second pattern is formation of one or more tumour masses. The least common pattern is the intravascular form where lymphoma cells fill all vascular components. Almost every histological lymphoma subtype may be encountered. Diffuse large B-cell lymphoma, including its variants, constitutes the single most frequent type of PRL and SRL {448,750, 755,2097,2647}. Prognosis and predictive factors Secondary renal lymphoma usually indicates stage IV disease with dismal prognosis {327,622,1267,2097}. In PRL, dissemination to extrarenal sites is common and confers a bad prognosis as well {622}. Modern radiochemotherapy has improved survival and renal functional compromise {2097,2696}. Fig. 1.129 Lymphoma. Neuroblastoma / Paraganglioma / Lymphomas 85
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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
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 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: Primitive neuroectodermal tumour (E
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
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
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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
Page 353 and 354:
Promoter methylation, 21 Prostate s
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