Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET

Mod Pathol. 2002 Jun;15(6):673-8. doi: 10.1038/modpathol.3880585.

Abstract

We describe two cases of peripheral primitive neuroectodermal tumor-Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase-polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.

Publication types

  • Case Reports
  • Review

MeSH terms

  • 12E7 Antigen
  • Antigens, CD / analysis
  • Cell Adhesion Molecules / analysis
  • Child
  • Chromosome Aberrations
  • Chromosomes, Human, Pair 22 / genetics
  • Diagnosis, Differential
  • Dura Mater / chemistry
  • Dura Mater / metabolism
  • Dura Mater / pathology*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Male
  • Meningeal Neoplasms / genetics
  • Meningeal Neoplasms / metabolism
  • Meningeal Neoplasms / pathology*
  • Neuroectodermal Tumors, Primitive, Peripheral / genetics
  • Neuroectodermal Tumors, Primitive, Peripheral / metabolism
  • Neuroectodermal Tumors, Primitive, Peripheral / pathology*
  • Neurofilament Proteins / analysis
  • S100 Proteins / analysis
  • Sarcoma, Ewing / genetics
  • Sarcoma, Ewing / metabolism
  • Sarcoma, Ewing / pathology*
  • Synaptophysin / analysis

Substances

  • 12E7 Antigen
  • Antigens, CD
  • CD99 protein, human
  • Cell Adhesion Molecules
  • Neurofilament Proteins
  • S100 Proteins
  • Synaptophysin