Abstract

Introduction

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. MPNSTs comprise approximately 5-10% of all soft tissue sarcomas. They can occur either spon-taneously or in association with neurofibromatosis-1.

The etiology is unknown but there is a higher incidence in patients with a history of radiation exposure. Up to 50% of MPNSTs occur in patients with NF1, demonstrating the tendency for this tumor to arise from a preexisting neurofibroma. Cross sectional studies have previously demonstrated a 1-2% prevalence of MPNST among NF1 patients although a recent study showed these patients have a 10% lifetime risk of ultimately developing an MPNST.