Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis--a pediatric case report and review of the literature.

Abstract:

:We report a 5 year-old boy with primary anaplastic pleomorphic xanthoastrocytoma (PXA) with whole neuroaxis dissemination at diagnosis who experienced the sudden onset of generalized convulsion. Head- and spinal magnetic resonance imaging (MRI) showed widespread gadolinium (Gd)-enhanced lesions extending to the bilateral frontal- and medial temporal lobes and the spinal cord. Subsequent MRI study demonstrated that the lesion size increased without any neurological deterioration. Under a histopathologic diagnosis of anaplastic PXA he underwent adjuvant chemotherapy consisting of 12 cycles of carboplatin and vincristine. The patient is alive without any neurological deficits; follow-up MRI showed that the lesions remained stable during 18 months of chemotherapy. We report a very rare pediatric case of primary anaplastic PXA with dissemination involving the entire neuroaxis at the time of diagnosis.

journal_name

J Neurooncol

authors

Okazaki T,Kageji T,Matsuzaki K,Horiguchi H,Hirose T,Watanabe H,Ohnishi T,Nagahiro S

doi

10.1007/s11060-009-9876-6

subject

Has Abstract

pub_date

2009-09-01 00:00:00

pages

431-7

issue

3

eissn

0167-594X

issn

1573-7373

journal_volume

94

pub_type

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