Current treatment options for malignant peripheral nerve sheath tumors.

Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas characterized by high risk of local recurrence and distant metastasis. The only known curative therapy is complete resection. Adjuvant radiation is recommended for larger lesions or those with more aggressive histology. Given the dismal prognosis in tumors that cannot be cured by surgery alone and the lack of systemic therapy with proven benefit, targeted therapies based on knowledge of activation of the Ras pathway and downstream effectors have been trialed in MPNST, thus far without proven benefit. However, novel or combination therapies based on recent preclinical advances are highly desirable and are the subject of ongoing clinical trials.