 | This article's factual accuracy is disputed. (March 2018) |
| Malignant peripheral nerve sheath tumor | |
|---|---|
| Other names | Malignant schwannoma,[1] Neurofibrosarcoma,[1] and Neurosarcoma[1] |
 | |
| Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern. H&E stain. | |
| Specialty | Neuro-oncology |
A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral 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 and often radiotherapy are done as adjuvant and/or neoadjuvant treatment depending upon various risk factors.
- ^ a b c Rapini RP, Bolognia JL, Jorizzo JL (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
- ^ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (May 2002). "Malignant peripheral nerve sheath tumours in neurofibromatosis 1". Journal of Medical Genetics. 39 (5): 311–314. doi:10.1136/jmg.39.5.311. PMC 1735122. PMID 12011145.