title: 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.
authors: Department of Pathology, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287-6417, USA.
authors: Aaron W
authors: AW
authors: James
authors: Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
authors: Elizabeth
authors: E
authors: Shurell
authors: Sarcoma Service, Division of Hematology/Oncology, University of California, Los Angeles, 2825 Santa Monica Boulevard, Suite 213 TORL, Santa Monica, CA 90404, USA.
authors: Arun
authors: A
authors: Singh
authors: Department of Pathology & Laboratory Medicine, University of California, Los Angeles, Box 951732, 13-145D CHS, Los Angeles, CA 90095-1732, USA.
authors: Sarah M
authors: SM
authors: Dry
authors: Division of Surgical Oncology, University of California, Los Angeles, 10833 LeConte Avenue, Room 54-140 CHS, Los Angeles, CA 90095-1782, USA. Electronic address: fceilber@mednet.ucla.edu.
authors: Fritz C
authors: FC
authors: Eilber
mesh_terms: D019788:Fluorodeoxyglucose F18; D006801:Humans; D018317:Nerve Sheath Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology*; D009442:Neurilemmoma / Q000150:complications* / Q000473:pathology; D009455:Neurofibroma / Q000150:complications* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D049268:Positron-Emission Tomography / Q000379:methods*; D012509:Sarcoma
chemical_list: D019788:Fluorodeoxyglucose F18
keywords: Atypical neurofibroma; Malignant peripheral nerve sheath tumor; NF1; Neurofibroma; Neurofibromatosis