Choroid plexus carcinomas and rhabdoid tumors: phenotypic and genotypic overlap

Five of six poorly differentiated choroid plexus carcinomas identified at our institution contained cells displaying a rhabdoid phenotype. Immunoperoxidase stains showed focal positivity for cytokeratin, epithelial membrane antigen, glial fibrillary acidic protein, S100, and vimentin. The MIB-1 prol...

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Veröffentlicht in:Pediatric and developmental pathology 2001-11, Vol.4 (6), p.545-549
Hauptverfasser: Wyatt-Ashmead, J, Kleinschmidt-DeMasters, B, Mierau, G W, Malkin, D, Orsini, E, McGavran, L, Foreman, N K
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Sprache:eng
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Zusammenfassung:Five of six poorly differentiated choroid plexus carcinomas identified at our institution contained cells displaying a rhabdoid phenotype. Immunoperoxidase stains showed focal positivity for cytokeratin, epithelial membrane antigen, glial fibrillary acidic protein, S100, and vimentin. The MIB-1 proliferative index ranged from 7.0% to 27.1%. All six tumors were p53 positive. Only the one child with Li-Fraumeni syndrome had a p53 germline mutation. Electron microscopy verified choroid plexus differentiation and the co-existence of rhabdoid cells. Of the five studied, four had deletions of chromosome 22 [three with monosomy 22 and one with del(22)(q12)]. Thus, there was a phenotypic and genotypic overlap between choroid plexus carcinomas and rhabdoid tumors.
ISSN:1093-5266
1615-5742
DOI:10.1007/s10024001-0085-3