Journal
JOURNAL OF NEURO-ONCOLOGY
Volume 84, Issue 2, Pages 217-222Publisher
SPRINGER
DOI: 10.1007/s11060-007-9361-z
Keywords
atypical teratoid; rhabdoid tumor; pleomorphic xanthoastrocytoma; FISH; immunohistochemistry; INI1; BAF47
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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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