title: Primary primitive neuroectodermal tumor of the spinal cord associated with neural tube defect.
abstract: Certain spinal-cord (SC) neoplasms, principally lipomas and dermoid tumors, have been diagnosed in association with characteristic neuroskeletal malformations thought to result from neural-tube defects (NTD). To our knowledge, no such association has been recognized for primary primitive neuroectodermal tumor of the SC (PNET-SC), an SC malignancy which has been reported in only 3 children and 5 adults. We describe here the occurrence and treatment results of PNET-SC in a boy who exhibited several neuroskeletal malformations suggestive of an underlying NTD. By undertaking a comparative analysis of the literature with respect to both the histology and the clinical presentation of this child's tumor, we document an occurrence of an uncommon malignancy, PNET-SC, and identify PNET-SC as another SC neoplasma which may be associated with NTD.
authors.affiliation: Department of Pediatrics, Wayne State University School of Medicine, Detroit, Mich.
authors.forename: D R
authors.initials: DR
authors.lastname: Freyer
authors.forename: R J
authors.initials: RJ
authors.lastname: Hutchinson
authors.forename: P E
authors.initials: PE
authors.lastname: McKeever
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000150:complications* / Q000628:therapy; D009436:Neural Tube Defects / Q000150:complications*; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000628:therapy