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Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are a rare variety of soft tissue
sarcoma of ectomesenchymal origin. MPNSTs arise from major or minor peripheral nerve
branches or sheaths of peripheral nerve fibers and are derived from Schwann cells
or pluripotent cells of neural crest origin. Arthur Purdy Stout played a pivotal role
in the development of our current understanding of the pathogenesis of peripheral
nerve sheath tumors by identifying the Schwann cell as the major contributor to the
formation of benign and malignant neoplasms of the nerve sheath. Although this fact
remains essentially true, the cell of origin of the MPNST remains elusive and has
not yet conclusively been identified. Some have suggested these tumors may have multiple
cell line origins. In the present review, MPNSTs and their epidemiology, diagnosis,
management, and treatment are discussed.