Cutaneous Malignant Peripheral Nerve Sheath Tumor

Surg Pathol Clin. 2017 Jun;10(2):337-343. doi: 10.1016/j.path.2017.01.008. Epub 2017 Mar 14.


Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of neuroectodermal origin arising in the dermis and/or subcutis. In contrast with their deep soft tissue and visceral counterparts, cutaneous MPNSTs are rarely associated with neurofibromatosis type 1. Two main subtypes of cutaneous MPNST can be distinguished histologically: conventional (ie, spindle cell) and epithelioid MPNST. The 2 subtypes also differ in predilection for deep versus superficial locations, association with preexistent benign peripheral nerve sheath tumors and S100 immunohistochemistry. Herein, we review current knowledge of cutaneous MPNST and discuss its differential diagnosis.

Keywords: Conventional variant; Epithelioid variant; Malignant peripheral nerve sheath tumor; Superficial/cutaneous.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunohistochemistry
  • Male
  • Neurilemmoma / metabolism
  • Neurilemmoma / pathology*
  • Neurofibromatosis 1 / pathology
  • Peripheral Nervous System Neoplasms / metabolism
  • Peripheral Nervous System Neoplasms / pathology*
  • S100 Proteins / metabolism
  • Sarcoma / metabolism
  • Sarcoma / pathology
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*


  • S100 Proteins