Primary primitive neuroectodermal tumor of the spinal cord associated with neural tube defect

Pediatr Neurosci. 1989;15(4):181-7. doi: 10.1159/000120466.

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abnormalities, Multiple*
  • Adult
  • Child
  • Combined Modality Therapy
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Neoplasms, Germ Cell and Embryonal / complications*
  • Neoplasms, Germ Cell and Embryonal / therapy
  • Neural Tube Defects / complications*
  • Spinal Cord Neoplasms / complications*
  • Spinal Cord Neoplasms / therapy