Malignant peripheral nerve sheath tumors

The rarity of malignant peripheral nerve sheath tumors (MPNSTs) and the lack of any singular diagnostic radiologic or pathologic signature lead to several management challenges. These tumors are best managed as part of a multidisciplinary team so as to optimize patient care and facilitate research. Suspicion of an MPNST based on clinical or radiologic alteration of a soft tissue mass in proximity to a peripheral nerve, especially in the context of neurofibromatosis type 1, should lead to referral to such a tertiary center. Early diagnosis followed by oncologic surgery to obtain tumor-free margins provides the best chance for long-term cure. Psychologic support and occupational rehabilitation are vital components of the overall care of these relatively young patients faced with often disabling surgery. Current adjuvant therapy with radiation and chemotherapy is suboptimal. There have been major inroads toward the molecular biologic understanding of MPNSTs, with several biologic targets that are of potential therapeutic interest. Proper evaluation of these novel and promising management strategies requires a concerted effort to refer these patients to the tertiary centers through which multi-institutional clinical trials can be undertaken.