Malignant peripheral nerve sheath tumor classification: Difference between revisions
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==Overview== | ==Overview== | ||
A '''malignant peripheral nerve sheath tumor''' (MPNST) or '''malignant neurolemmoma''' is a form of [[cancer]] of the [[connective tissue]] surrounding [[nerve]]s. 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 [[radiation therapy|radiotherapy]] are done as adjuvant and/or neoadjuvant treatment. | |||
==References== | ==References== |
Revision as of 05:20, 23 January 2012
Malignant peripheral nerve sheath tumor Microchapters |
Differentiating Malignant Peripheral Nerve Sheath Tumor from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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.