A malignant peripheral nerve sheath tumor (MPNST) or malignant neurolemmoma 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 diagonosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13% (Evans et al 2002). The first-line treatmenet is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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