title: 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.
authors.affiliation: Department of Neurosurgery, School of Medicine, The University of Tokushima, Tokushima, Japan. tokazaki1978@sirius.ocn.ne.jp
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Okazaki
authors.forename: Teruyoshi
authors.initials: T
authors.lastname: Kageji
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Matsuzaki
authors.forename: Hidehisa
authors.initials: H
authors.lastname: Horiguchi
authors.forename: Takanori
authors.initials: T
authors.lastname: Hirose
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Watanabe
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Ohnishi
authors.forename: Shinji
authors.initials: S
authors.lastname: Nagahiro