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Tumores Neuroendocrinos Del Tracto Urinario

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Tumores Neuroendocrinos Del Tracto Urinario Tumores Neuroendocrinos del Tracto Urinario Antonio López-Beltrán Córdoba Introduction • Three basic types of neuroendocrine (NE) tumours with diverse clinicopathological features and outcome are identified in the urinary system and male genital organs: • Carcinoid tumour (typical and atypical) • Small cell carcinoma (SCC) • Large cell NE carcinoma (LCNEC), • Morphologically, histochemically, immunohistochemically and ultrastructurally similar to its counterpart in other organs, such as lung or gastrointestinal tract. • Metastases can be detected at the initial evaluation, although they have been reported up to several years after removal (carcinoid), emphasizing the need for a long-term follow-up. • Although the occurrence is rare, SCC and LCNEC are highly aggressive. Introduction • Neuroendocrine (NE) tumours represent a heterogeneous group of neoplasms originating from NE cells. • Such cells are either present in endocrine organs or dispersed through the body, and produce neurotransmitters, neuromodulators or neuropeptide hormones. • The NE cells are defined immunohistochemically by the presence in the cytoplasm of markers, such as chromogranin A and neurone- specific enolase (NSE) • Show dense-core granules at the ultrastructural level. • The classification of the NE tumours largely depends upon the anatomical site and organ of origin. In the lung NE tumours include four groups of neoplasms with diverse prognosis – Typical and atypical carcinoid, large-cell NE carcinoma (LCNEC) and small cell carcinoma (SCC). Introduction • Potential origins of the NE neoplasms of the urinary system and male genital organs are: • (i) Derivation from NE cells of the diffuse NE system, e.g. those identified in the normal urothelial tract and prostate and which might increase in number in reactive or metaplastic settings • (ii) Derivation from a multipotent stem cell, a concept crucial to understanding the nature of NE tumours arising in conjunction with epithelial or germ cell malignancies, which might express markers of both components. • (iii) When admixed with other carcinomas, both components are usually clonally related • The role of the uropathologist is to establish the diagnosis and identify features of prognostic importance. Based on such an information, the urologist/oncologist will select the follow-up procedure and therapeutic options in each patient. A close collaboration between the pathologist and urologist/oncologist is also of paramount importance to exclude a metastatic lesion from more common sites. Tumores Neuroendocrinos del Tracto Urinario • Carcinoma de células pequeñas • Carcinoma neuroendocrino de células grandes. • Carcinoide: Típico o Atípico – Carcinoide típico: Carcinoma (tumor) neuroendocrino bien diferenciado – Carcinoide atípico: Carcinoma neuroendocrino moderadamente diferenciado • Vejiga, Próstata, Riñón, Testículo – Muy ocasionales casos en pelvis renal, pene o uretra Small Cell Carcinoma • Variant histology in the bladder tumor: • Key factor<< risk stratification • All of the variant histologies portend a worse prognosis than pure urothelial carcinoma. • The most common aberrant differentiation patterns and their incidences are: • Squamous and/or glandular differentiation in 10% to 60% of patients • Small-cell carcinoma in 0.5% to 1.2% • Micropapillary carcinoma in 0.7% • Sarcomatoid carcinoma in 0.2% Small cell carcinoma • Small cell carcinoma<<malignant • Patients with organ-confined cancers neuroendocrine neoplasm derived from had marginally better survival than the urothelium << mimics its pulmonary those with non organ-confined cancer counterpart. (P=0.06). • 64 cases<< all had muscle-invasive At histology<<small, rather disease at presentation. uniform cells, with nuclear • 38 patients (59%) underwent molding, scant cytoplasm cystectomy • 66% of patients had metastases and inconspicuous nucleoli. <<regional LN, bone, liver and lung. Mitoses may be frequent. • 20 (32%) were pure small cell Ca Necrosis is common and • 44 cases (68%) small cell Ca with: there may be DNA • urothelial carcinoma, 35 cases • adenocarcinoma, 4 cases encrustation of blood • sarcomatoid urothelial Ca, 2 cases vessels walls (Azzopardi • 3 cases with both adenoca and UC phenomenon). • Supervivencia 16% a 3 años Vascular invasion common Diagnosis>> HE Cancer, 2004 P=0.06 Small cell carcinoma Immunohistochemistry IHC: NSE ChG Synapto TTF-1 High ki67 Others: CD56, CD57 c-kit 30%. EGFR 30% Diagnosis>> HE TTF1SCC UB: 25-39% C-kit 27%+ CCR, 2008 - Láser microdisectión - Chromosome X Inactivación (Humara Test) -LOH Concordant genetic alterations thus suggesting that urothelial component and SCC both originate from the same cell in the urothelium Small cell Ca of the bladder DD • High grade poorly differentaited TCC and PCA • Solid, small cell variant of alveolar rhadomyosarcoma • PNET • Bladder Lymphoma • Carcinoid/Atypical carcinoid/Moderate-well differentiated NE carcinoma Appl Immunohistochem Mol Morphol. 2011 Oct;19(5):395-9. Distinction of high-grade neuroendocrine carcinoma/small cell carcinoma from conventional urothelial carcinoma of urinary bladder: an immunohistochemical approach. Thompson S, Cioffi-Lavina M, Chapman-Fredricks J, Gomez- • RESULTS: Fernandez C, Fernandez-Castro G, Jorda M. • Pan-cytokeratin was positive in 6 of 7 cases (86%) of the HGNEC/SmCC group. • All 7 tumors were positive for synaptophysin, 6 of them were negative for p63 and chromogranin, and 1 was positive for p63 and chromogranin. • All 21 high-grade UC with neuroendocrine-like pattern of growth showed positive staining for pan-cytokeratin, and were all negative for synaptophysin and chromogranin. • Sixteen (76%) of high-grade UC were also positive for p63. • All 11 mixed tumors were positive for pan-cytokeratin. In 10 of the 11 mixed tumors (91%), synaptophysin was positive in the neuroendocrine differentiated areas and it was negative in the urothelial component. CONCLUSIONS: • A limited immunohistochemical panel including pan- cytokeratin, synaptophysin, and p63 discriminates HGNEC/SmCC from high-grade UC. From Amin MB: Solid, small cell variant of alveolar rhadomyosarcoma Prostate SCC • Clinical features • SCC represents 1–5% of all prostatic malignancies when mixed adenocarcinoma-NEC are included. • Many patients have a previous history of a hormonally treated adenocarcinoma. • As the SCC component predominates, serum PSA levels decline and might be undetectable. • Most NECs of the prostate lack clinically evident hormone production. • Origins • The presence of mixed prostatic acinar adenocarcinoma in many NECs, coupled with evidence of cells that might co-express PSA, PAP, androgen receptor and NE markers suggests the evolution of a subset of multipotent non-NE prostatic tumour cells as the derivation for prostatic high-grade NEC. • As in NEC of the urothelium, local extension or distant spread of NEC from other sites must be excluded clinically, as the morphological and immunohistochemical features might be identical. Prostate SCC • Morphology and immunophenotypic features • Histologically, SCC is identical to SCC of the lung. • In about half the cases the tumours are mixed SCC and adenocarcinoma of the prostate. • Diagnostically, NEC should not be assigned a Gleason grade and must be differentiated from diffuse growth of Gleason pattern 5 prostate adenocarcinoma by the large cells with lower nuclear/chromatin ratio, prominent nucleoli, and absent nuclear moulding seen in the latter. • There are conflicting studies as to whether SCCs of the prostates are positive for TTF-1, to distinguish them from a metastasis from the lung. A recent study showed that stains for TTF-1 are positive in 52.3% of cases . The same study also showed that P504S/a- methylacyl CoA racemase and prostate-specific membrane antigen are better for identifying the prostatic origin of SCC than PSA, although most (60%) prostatic SCCs are negative for all three markers. • Prognosis • The mean survival is < 1 year; there is no difference in prognosis between patients with pure SCC and those with mixed glandular and SCCs. Antibody Small cell carcinoma Poorly differentiated (approximate adenocarcinoma percentage of (approximate percentage positivity) of positivity) Cytokeratin (94%) + (70%) Cytokeratin high (35%) −/+ − (0-33%) molecular weight CAM 5.2 (72%) + (90%) CK 7 (39%) −/+ −/+ (30%) CK 20 (11%) −/+ −/+ (10%) PSA (24%) −/+ ++ (85%) PSMA (20%) −/+ ++ (90%) PSAP (22%) −/+ ++ (95%) P501s (25%) −/+ ++ (90%) p63 (40%) −/+ −−/+ (15%) TTF1 (83%) +/− − (10%) CD 56 (92%) + − (10%) Chromogranin (80%) + − (10%) Synaptophysin (85%) + −/+ (13%) CD44 (60-96%) ++ − (5%) Kidney SCC Clinical features • The mean age of the patients with SCC is 59 years, with no sex predilection. • Most tumours are located close to the renal pelvis • The left kidney is more frequently involved (left/right ratio, 1 : 1.5). • Abdominal pain and gross haematuria are the most frequent clinical symptoms. • Origins • In most cases SCC of the kidney at least partly invades the renal pelvis and occasionally coexists with an in situ and/or a papillary urothelial carcinoma. • It probably originates from multipotent urothelial (stem) cells capable of multidirectional differentiation or from conventional high-grade transitional cells through a process of metaplasia. • An origin from intrinsic NE cells of the normal urinary tract is less likely. kidney • Morphology and immunophenotypic features • Macroscopically, the tumour presents as a soft, whitish, gritty and necrotic renal mass, often extending
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