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

被引：59

作者：

Chacko, Geeta

Chacko, Ari G.

Dunham, Christopher P.

Judkins, Alexander R.

Biegel, Jaclyn A.

Perry, Arie

机构：

[1] Washington Univ, Sch Med, Div Neuropathol, St Louis, MO 63110 USA

[2] Christian Med Coll & Hosp, Dept Neurol Sci, Div Neuropathol, Vellore 632004, Tamil Nadu, India

[3] Christian Med Coll & Hosp, Dept Neurol Sci, Div Neurosurg, Vellore 632004, Tamil Nadu, India

[4] Univ Penn, Childrens Hosp Philadelphia, Sch Med, Dept Pathol, Philadelphia, PA 19104 USA

[5] Univ Penn, Childrens Hosp Philadelphia, Sch Med, Dept Pediat, Philadelphia, PA 19104 USA

来源：

JOURNAL OF NEURO-ONCOLOGY | 2007年 / 84卷 / 02期

关键词：

atypical teratoid; rhabdoid tumor; pleomorphic xanthoastrocytoma; FISH; immunohistochemistry; INI1; BAF47;

D O I：

10.1007/s11060-007-9361-z

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：217 / 222

页数：6

共 26 条

[1] Atypical teratoid/rhabdoid tumor evolving from an optic pathway ganglioglioma: Case study [J].