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01-09-2007 | Clinical Study - Patient Studies

Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma

Authors: Geeta Chacko, Ari G. Chacko, Christopher P. Dunham, Alexander R. Judkins, Jaclyn A. Biegel, Arie Perry

Published in: Journal of Neuro-Oncology | Issue 2/2007

Abstract

We present a case of a 23-year-old man with a tumor containing glial and rhabdoid elements where the former had features of a pleomorphic xanthoastrocytoma (PXA) and the latter had the immunophenotype and genetic profile of an atypical rhabdoid/teratoid tumor. The patient presented with a short history of raised intracranial pressure with rapid deterioration in sensorium. He had a poor outcome despite surgery and radiotherapy. We report this case because of its unusual presentation in adulthood and its occurrence in association with a PXA. We speculate that the PXA was a quiescent tumor and that the secondary genetic alterations, including inactivation of the INI1 gene led to clinical progression.

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Title: Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma
Authors: Geeta Chacko
Ari G. Chacko
Christopher P. Dunham
Alexander R. Judkins
Jaclyn A. Biegel
Arie Perry
Publication date: 01-09-2007
Publisher: Kluwer Academic Publishers-Plenum Publishers
Published in: Journal of Neuro-Oncology / Issue 2/2007
Print ISSN: 0167-594X
Electronic ISSN: 1573-7373
DOI: https://doi.org/10.1007/s11060-007-9361-z

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Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma

Abstract

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