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Gliosarkóm s komponentou pripomínajúcou alveolárny rabdomyosarkóm: popis prípadu s doposiaľ nepopísanou sarkómovou zložkou


Authors: M. Švajdler jr. 1;  B. Rychlý 2;  M. Gajdoš 3;  F. Pataky 3;  L. Fröhlichová 1;  A. Perry 4
Authors‘ workplace: Department of pathology UNLP Košice, Slovakia 1;  Cytopathos, spol. s. r. o., Bratislava, Slovakia 2;  Department of neurosurgery UNLP and P. J. Safarik University, Faculty of Medicine Košice, Slovakia 3;  Department of Pathology, Division of Neuropathology, University of California, San Francisco, USA 4
Published in: Čes.-slov. Patol., 48, 2012, No. 4, p. 210-214
Category: Original Articles

Overview

Gliosarkóm (GS) je relatívne vzácny variant glioblastómu, charakterizovaný bifázickou gliálnou a mezenchymálnou diferenciáciou. Sarkomatózna časť najčastejšie pripomína fibrosarkóm alebo tzv. malígny fibrózny histiocytóm. Vzácne je v GS prítomná heterológna diferenciácia vo forme osteosarkómu, chondrosarkómu, liposarkómu, leiomyosarkómu, skvamóznej alebo žľazovej malígnej epiteliálnej diferenciácie, alebo diferenciácie pripomínajúcej primitívny neuroektodermálny tumor (PNET). Keď je v GS prítomná rabdomyosarkómová diferenciácia, je vo forme malígnych vretenovitých buniek s priečne pruhovanými bunkami alebo okrúhlymi rabdomyoblastami, pripomínajúca embryonálny rabdomyosarkóm. V kazuistike popisujeme GS s komponentou pripomínajúcou alveolárny rabdomyosarkóm. Nádor rástol v solídnych a alveolárnych formáciách a bol zložený z nediferencovaných primitívnych malých okrúhlych buniek s minimálnou cytoplazmou, nápadne zvýšenou mitotickou aktivitou a početnými apoptózami. Rabdomyosarkomatózna diferenciácia bola potvrdená pozitívnou imunohistochemickou reakciou na dezmín a myogenín. Podľa naších vedomostí, takýto histologický vzor nebol v GS doposiaľ popísaný. V krátkosti je prebraná diferenciálna diagnóza prípadu.

Kľúčové slová:
gliosarkóm – alveolárny rabomyosarkóm – myogenín - desmín


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