Ewing's family of tumors involving structures related to the central nervous system: a review

Pediatr Dev Pathol. May-Jun 2000;3(3):203-10. doi: 10.1007/s100249910026.


This review consolidates information gleaned from several case reports and larger series on Ewing's sarcoma family of tumors (EFT) involving structures related to and found in the central nervous system (CNS). These tumors involve the skull, the spinal column, adjacent soft tissues, the meninges, and the brain. We have separated the cases by skull region and spinal column level, and we discuss the attendant differences in prognosis following treatment by neurosurgery, radiation, and chemotherapy. Light and electron microscopic features can be used to differentiate EFT from other small round blue cell tumors that involve the CNS (central primitive neuroectodermal tumor, lymphoma, etc.). Recent molecular and genetic findings in EFT provide new diagnostic methods. We conclude that EFT involving the CNS and adjacent structures is not so rare as previously stated and that the prognosis is more favorable, as a rule, than for the more common examples arising in the long bones and pelvis.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / secondary
  • Central Nervous System Neoplasms / therapy
  • Child
  • Diagnosis, Differential
  • Epidural Neoplasms / diagnosis
  • Female
  • Genetic Techniques
  • Humans
  • Immunohistochemistry
  • Infant
  • Jaw Neoplasms / diagnosis
  • Male
  • Microscopy, Electron
  • Neuroectodermal Tumors, Primitive / diagnosis
  • Prognosis
  • Sarcoma, Ewing / diagnosis*
  • Sarcoma, Ewing / secondary
  • Sarcoma, Ewing / therapy
  • Skull Neoplasms / diagnosis
  • Soft Tissue Neoplasms / diagnosis
  • Soft Tissue Neoplasms / secondary
  • Spinal Neoplasms / diagnosis