Malignant peripheral nerve sheath tumor overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
A malignant peripheral nerve sheath tumor (MPNST) 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.
A malignant peripheral nerve sheath tumor (MPNST) (also known as "malignant schwannoma,"[1] "neurofibrosarcoma,"[1] and "neurosarcoma"[1]) 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%.[2] MPNST with rhabdomyoblastomatous component are called malignant triton tumors.
The first-line treatment is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.
References
- ↑ 1.0 1.1 1.2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (May 2002). "Malignant peripheral nerve sheath tumours in neurofibromatosis 1". J. Med. Genet. 39 (5): 311–4. doi:10.1136/jmg.39.5.311. PMC 1735122. PMID 12011145.