Back Tumor maligne de la beina del nervi perifèric Catalan Maligní nádor z pochvy periferního nervu Czech Maligner peripherer Nervenscheidentumor German Tumor maligno de la vaina del nervio periférico Spanish Pahanlaatuinen hermotuppikasvain Finnish Złośliwy nowotwór osłonek nerwów obwodowych Polish Malignant peripheral nerve sheath tumor SIMPLE Злоћудни рак омотача периферних нерава Serbian
 | 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.