@article{3128155, title = "Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus", author = "Alexiou, G.A. and Siozos, G. and Stefanaki, K. and Moschovi, M. and Prodromou, N.", journal = "Journal of Child Neurology", year = "2013", volume = "28", number = "2", pages = "246-250", issn = "0883-0738, 1708-8828", doi = "10.1177/0883073812441061", keywords = "beta tubulin; CD99 antigen; cytokeratin 8; epithelial membrane antigen; glial fibrillary acidic protein; nuclear protein; protein bcl 2; protein p53; synaptophysin, article; brain ventricle peritoneum shunt; case report; cervical spine; disease association; fetus echography; follow up; hemiparesis; human; hydrocephalus; immunohistochemistry; infant; male; neuroectoderm tumor; nuclear magnetic resonance imaging; priority journal; protein expression; radical resection; spinal cord tumor; tumor cell; vertebra, Chromosomal Proteins, Non-Histone; DNA-Binding Proteins; Humans; Hydrocephalus; Infant; Magnetic Resonance Imaging; Male; Nerve Tissue Proteins; Neuroectodermal Tumors, Primitive; Spinal Cord; Spinal Cord Neoplasms; Transcription Factors", 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. © The Author(s) 2013." }