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  • Title: Malignant Peripheral Nerve Sheath Tumor.
    Author: James AW, Shurell E, Singh A, Dry SM, Eilber FC.
    Journal: Surg Oncol Clin N Am; 2016 Oct; 25(4):789-802. PubMed ID: 27591499.
    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.
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