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A malignant peripheral nerve sheath tumor is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.
The first-line treatment is surgical resection with wide margins. Chemotherapy and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.
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