Malignant peripheral nerve sheath tumor

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). Treatment is with chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy.

References

• Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002;39:311-4. PMID 12011145.
• Pathology of Malignant Nerve Sheath Tumour