DOI: 10.36347/sjmcr.2017.v05i05.007

Pages: 317-320

: Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma of ectomesenchymal origin. It is the malignant counterpart of benign soft tissue tumors like neurofibromas and schwannomas and may often follow them. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. MPNSTs have an incidence of 1 per 1,000,000 people per year and comprise around 3% of soft-tissue sarcomas. Tumours arise from Schwann cells or perineural cells that ensheath axons and extend along neural tracts. They are aggressive cancers that have high rates of recurrence and a poor prognosis.