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Malignant peripheral nerve sheath tumor

The clinical spectrum and outcome of treatment

From the Department of Neurology (Drs. Baehring and Batchelor), Massachusetts General Hospital, Harvard Medical School, and Department of Biostatistics (Dr. Betensky), Harvard School of Public Health, Boston, MA.

Address correspondence and reprint requests to Dr. T.T. Batchelor, Brain Tumor Center, Massachusetts General Hospital, Cox 315, 100 Blossom St., Boston, MA 02114; e-mail: tbatchelor{at}partners.org

Malignant peripheral nerve sheath tumors (MPNST) are derived from Schwann cells or pluripotent cells of the neural crest. Delay of diagnosis is common, especially in lesions affecting proximal parts of the peripheral nervous system. Presented is a series of 54 patients with MPNST seen at a single institution over a 10-year period. In this series, tumor diameter of <5 cm, gross total resection of the tumor, and younger age were favorable prognostic variables.