Renal Cell Carcinoma With Rhabdoid Features

摘要: Neoplasms with rhabdoid features have been reported at many anatomic sites. In the kidney, tumors are typically found in children, whereas only rare examples adults. Little is known of renal cell carcinomas (RCCs) that exhibit features. The objective this study was to determine incidence RCC attributes and characterize histologic, immunophenotypic, ultrastructural by retrospective analysis 480 consecutively identified cases radical nephrectomy specimens. Immunohistochemical evaluation performed foci using a panel antibodies pancytokeratin (pan-CK), CK7, CK20, epithelial membrane antigen (EMA), S-100 protein, desmin, vimentin, neuron-specific enolase (NSE), muscle-specific actin (MSA), smooth muscle (SMA), human melanoma, black-45 (HMB-45), glial fibrillary acidic protein (GFAP). Electron microscopy also selected cases. presence extent relation pathologic stage grade were assessed. Twenty-three (4.7%) exhibited 23 patients all adults mean age 61.8 years (age range, 33-84 yrs). Fifteen men eight women. Histologically, typified sheets clusters variably cohesive, large epithelioid cells vesicular often eccentric nuclei, prominent nucleoli, large, paranuclear intracytoplasmic hyaline globules (inclusions). these related high histologic Fuhrman nonrhabdoid carcinoma component, an 0 84 I cases, 300 2 (2.6%), six 70 3 (8.9%), nine 26 4 (34.6%; p = x 10(-9)). grade. higher carcinomas. A total 52% (12 23) extrarenal extension compared 28% (24 92) contemporary RCCs without (p 0.03). size component ranged from 1 mm more than cm comprised 1% 50% mass. Immunoreactivity for vimentin (100%), NSE (79%), panCK (56%) present majority Substantial percentages immunopositive EMA (47%) (37%), minimal no immunohistochemical reactivity CK7 (5%), SMA CK20 (0%), desmin MSA HMB-45 GFAP (O%). distinctive globular, reaction pattern cytokeratin, EMA, immunostains. Ultrastructurally, had intermediate filament aggregates or condensation organelles, associated peripheral vacuolization. Adult may harbor neoplasms can be regarded as "composite" tumors. Rhabdoid elements important identify because their high-grade nature, association stage. should distinguished pure light clinicopathologic differences. differentiation adult represent clonal divergence and/ evolution, emergence particularly aggressive element.