title: Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus.
abstract: Spinal primitive neuroectodermal tumors are exceedingly rare. Herewith, we present the first case of an intramedullary spinal cord tumor associated with hydrocephalus in a 2-month-old boy that presented with left hemiparesis. The patient had been diagnosed on prenatal ultrasound with enlarged ventricular system. At his current admission, a brain magnetic resonance imaging (MRI) revealed hydrocephalus and an intramedullary lesion extending from the second cervical to the first thoracic vertebrae. Dissemination of the tumor was revealed intracranially and in the spinal canal. After a ventriculoperitoneal shunt placement a radical resection of the tumor was performed, however some small tumor remnants could not be safely removed. Postoperative there was no neurologic deterioration. The tumor was diagnosed as a central nervous system primitive neuroectodermal tumor (World Health Organization grade IV). Spinal intramedullary primitive neuroectodermal tumors are extremely rare. In such rare tumors, multiinstitutional studies are needed for treatment guidelines to be established.
authors.affiliation: Department of Neurosurgery, Children's Hospital Agia Sofia, Athens, Greece. alexiougrg@yahoo.gr
authors.forename: George A
authors.initials: GA
authors.lastname: Alexiou
authors.forename: George
authors.initials: G
authors.lastname: Siozos
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Stefanaki
authors.forename: Maria
authors.initials: M
authors.lastname: Moschovi
authors.forename: Neofytos
authors.initials: N
authors.lastname: Prodromou
mesh_terms: D002868:Chromosomal Proteins, Non-Histone / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000378:metabolism; D006801:Humans; D006849:Hydrocephalus / Q000150:complications* / Q000175:diagnosis; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009419:Nerve Tissue Proteins / Q000378:metabolism; D018242:Neuroectodermal Tumors, Primitive / Q000150:complications* / Q000175:diagnosis; D000071796:SMARCB1 Protein; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000175:diagnosis; D014157:Transcription Factors / Q000378:metabolism
chemical_list: D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D009419:Nerve Tissue Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors