title: Intracranial peripheral primitive neuroectodermal tumor manifesting as abducens nerve palsy.
abstract: BACKGROUND
  Peripheral primitive neuroectodermal tumor (PNET) occurring as an extra-axial lesion within the intracranial space and extending to the subarachnoid space is extremely rare.
  
  
  METHODS
  An 18-month-old girl presented with an intracranial peripheral PNET manifesting as abducens nerve palsy. Magnetic resonance imaging on admission revealed a lesion affecting the trigeminal and abducens nerves. The tumor was partially removed via the subtemporal approach. Histological examination showed a high-grade, undifferentiated neoplasm of small cell type with positive immunostaining for MIC2. The histological diagnosis was peripheral PNET.
  
  
  RESULTS
  Craniospinal radiotherapy reduced the tumor size, but adjuvant chemotherapy designed for Ewing's sarcomas and PNETs was not effective. She died 1 month after the last chemotherapy, despite whole craniospinal irradiation (total dose 53.2 Gy) and chemotherapy.
authors.affiliation: Department of Neurosurgery, Juntendo University Hospital, Hongo, Tokyo, Japan. horin@med.juntendo.ac.jp
authors.forename: N
authors.initials: N
authors.lastname: Horinaka
authors.forename: Y
authors.initials: Y
authors.lastname: Ito
authors.forename: M
authors.initials: M
authors.lastname: Miyajima
authors.forename: M
authors.initials: M
authors.lastname: Hishii
authors.forename: K
authors.initials: K
authors.lastname: Suzuki
authors.forename: M
authors.initials: M
authors.lastname: Saito
authors.forename: H
authors.initials: H
authors.lastname: Arai
mesh_terms: D000010:Abducens Nerve / Q000473:pathology; D020434:Abducens Nerve Diseases / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D001932:Brain Neoplasms / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D018242:Neuroectodermal Tumors, Primitive / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D015800:Protozoan Proteins / Q000378:metabolism
chemical_list: D015800:Protozoan Proteins; C069428:thrombospondin-related adhesive protein, protozoan