Malignant Peripheral Nerve Sheath Tumor

Aaron W. James, Elizabeth Shurell, Arun Singh, Sarah M. Dry, Fritz C. Eilber

Research output: Contribution to journalReview articlepeer-review

46 Scopus citations


Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common type of soft tissue sarcoma. Most MPNSTs arise in association with a peripheral nerve or preexisting neurofibroma. Neurofibromatosis type is the most important risk factor for MPNST. Tumor size and fludeoxyglucose F 18 avidity are among the most helpful parameters to distinguish MPNST from a benign peripheral nerve sheath tumor. The histopathologic diagnosis is predominantly a diagnosis of light microscopy. Immunohistochemical stains are most helpful to distinguish high-grade MPNST from its histologic mimics. Current surgical management of high-grade MPNST is similar to that of other high-grade soft tissue sarcomas.

Original languageEnglish (US)
Pages (from-to)789-802
Number of pages14
JournalSurgical Oncology Clinics of North America
Issue number4
StatePublished - Oct 1 2016


  • Atypical neurofibroma
  • Malignant peripheral nerve sheath tumor
  • NF1
  • Neurofibroma
  • Neurofibromatosis

ASJC Scopus subject areas

  • Surgery
  • Oncology


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