Erschienen in:
01.09.2007 | Clinical Study - Patient Studies
Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma
verfasst von:
Geeta Chacko, Ari G. Chacko, Christopher P. Dunham, Alexander R. Judkins, Jaclyn A. Biegel, Arie Perry
Erschienen in:
Journal of Neuro-Oncology
|
Ausgabe 2/2007
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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.