Malignant peripheral nerve sheath tumor classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Classification
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.[1]
Classification
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.[2]
References
- ↑ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (2002). "Malignant peripheral nerve sheath tumours in neurofibromatosis 1". Journal of Medical Genetics. 39 (5): 311–4. PMC 1735122. PMID 12011145. Retrieved 2012-09-11. Unknown parameter
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ignored (help) - ↑ Panigrahi, S.; Mishra, S.; Das, S.; Dhir, M. (2013). "Primary malignant peripheral nerve sheath tumor at unusual location". Journal of Neurosciences in Rural Practice. 4 (5): 83. doi:10.4103/0976-3147.116480. PMC 3808069. PMID 24174807.