Malignant Peripheral Nerve Sheath Tumor
- PMID: 27591499
- DOI: 10.1016/j.soc.2016.05.009
Malignant Peripheral Nerve Sheath Tumor
Abstract
Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common type of soft tissue sarcoma. Most MPNSTs arise in association with a peripheral nerve or preexisting neurofibroma. Neurofibromatosis type is the most important risk factor for MPNST. Tumor size and fludeoxyglucose F 18 avidity are among the most helpful parameters to distinguish MPNST from a benign peripheral nerve sheath tumor. The histopathologic diagnosis is predominantly a diagnosis of light microscopy. Immunohistochemical stains are most helpful to distinguish high-grade MPNST from its histologic mimics. Current surgical management of high-grade MPNST is similar to that of other high-grade soft tissue sarcomas.
Keywords: Atypical neurofibroma; Malignant peripheral nerve sheath tumor; NF1; Neurofibroma; Neurofibromatosis.
Copyright © 2016 Elsevier Inc. All rights reserved.
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