Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis--a pediatric case report and review of the literature.
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.
Okazaki
Toshiyuki
T
Department of Neurosurgery, School of Medicine, The University of Tokushima, Tokushima, Japan. tokazaki1978@sirius.ocn.ne.jp
Kageji
Teruyoshi
T
Matsuzaki
Kazuhito
K
Horiguchi
Hidehisa
H
Hirose
Takanori
T
Watanabe
Hiroyoshi
H
Ohnishi
Toshihiro
T
Nagahiro
Shinji
S