Malignant perineurioma (malignant peripheral nerve sheath tumor with perineural differentiation)

Clin Neuropathol. 2012 Nov-Dec;31(6):424-9. doi: 10.5414/NP300423.


The great majority of malignant peripheral nerve sheath tumors (MPNST) exhibit Schwannian differentiation. In recent years, a subset of perineurial MPNST (malignant perineurioma) has been identified based on their histologic, immunohistochemical and ultrastructural features. Immunopositivity for epithelial membrane antigen (EMA), glut-1 and claudin-1, is characteristic. Such tumors must be distinguished from benign perineurioma and a variety of atypical or malignant soft tissue tumors featuring EMA positivity. Herein, we report a perineurial MPNST involving the buttock of a 42-year-old woman. Nerve involvement was noted. The clinicopathologic features of reported examples are ummarized and key differential diagnoses are discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Buttocks / pathology
  • Female
  • Humans
  • Immunohistochemistry
  • Neoplasm Recurrence, Local / metabolism
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy
  • Nerve Sheath Neoplasms / metabolism
  • Nerve Sheath Neoplasms / pathology*
  • Nerve Sheath Neoplasms / therapy
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / pathology*
  • Soft Tissue Neoplasms / therapy