Malignant peripheral nerve sheath tumor

Malignant peripheral nerve sheath tumor
Updated: 08/07/2020
© Jun Wang, MD, PhD

General features
  • AKA: Malignant schwannoma, neurofibrosarcoma
  • Commonly associated with neurofibromatosis (NF) 1
  • May arise de novo
  • More common in adults
  • May have history of radiation exposure
  • May recur, distant metastases common
Clinical presentations
  • Deep seated tumor associated with major nerves
  • Commonly location: Neck, forearm, lower leg, buttock
Key morphological features
Markers
  • Positive for S-100, CD56
  • Negative for EMA, keratin (different from synovial sarcoma)
Treatment
  • Excision
  • Radiation
  • Chemotherapy


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