Malignant Peripheral Nerve Sheath Tumor

Aaron W James; Elizabeth Shurell; Arun Singh; Sarah M Dry; Fritz C Eilber

doi:10.1016/j.soc.2016.05.009

Malignant Peripheral Nerve Sheath Tumor

Surg Oncol Clin N Am. 2016 Oct;25(4):789-802. doi: 10.1016/j.soc.2016.05.009.

Authors

Aaron W James¹, Elizabeth Shurell², Arun Singh³, Sarah M Dry⁴, Fritz C Eilber⁵

Affiliations

¹ Department of Pathology, Johns Hopkins University, 600 North Wolfe Street, Baltimore, MD 21287-6417, USA.
² Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
³ Sarcoma Service, Division of Hematology/Oncology, University of California, Los Angeles, 2825 Santa Monica Boulevard, Suite 213 TORL, Santa Monica, CA 90404, USA.
⁴ Department of Pathology & Laboratory Medicine, University of California, Los Angeles, Box 951732, 13-145D CHS, Los Angeles, CA 90095-1732, USA.
⁵ 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.

PMID: 27591499
DOI: 10.1016/j.soc.2016.05.009

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.

Publication types

Review

MeSH terms

Fluorodeoxyglucose F18
Humans
Nerve Sheath Neoplasms / diagnostic imaging*
Nerve Sheath Neoplasms / pathology*
Neurilemmoma / complications*
Neurilemmoma / pathology
Neurofibroma / complications*
Neurofibroma / pathology
Neurofibromatosis 1 / complications
Neurofibromatosis 1 / diagnostic imaging*
Neurofibromatosis 1 / pathology
Positron-Emission Tomography / methods*
Sarcoma

Substances

Fluorodeoxyglucose F18