Malignant Peripheral Nerve Sheath Tumor Overview

A malignant peripheral nerve sheath tumor 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% . The first-line treatmenet is surgical resection with wide margins. Chemotherapy and often radiotherapy are...