Published in:
01-09-2009 | Case Report
Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis––a pediatric case report and review of the literature
Authors:
Toshiyuki Okazaki, Teruyoshi Kageji, Kazuhito Matsuzaki, Hidehisa Horiguchi, Takanori Hirose, Hiroyoshi Watanabe, Toshihiro Ohnishi, Shinji Nagahiro
Published in:
Journal of Neuro-Oncology
|
Issue 3/2009
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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.