Extended remission of a recurrent median nerve malignant peripheral nerve sheath tumor after multimodal treatment

Malignant peripheral nerve sheath tumors (MPNSTs) are difficult to control despite aggressive treatment. In this report the authors describe the treatment and follow-up review of a patient with neurofibromatosis Type 1 who harbored a recurrent median nerve MPNST. The man underwent preoperative intraarterial and intravenous chemotherapy followed by additional surgery for gross-total removal and postoperative radiotherapy. Two courses of preoperative intraarterial cisplatin and intravenous Adriamycin produced significant tumor shrinkage. Gross-total removal of the remaining tumor without amputation of the arm was followed by fractionated radiotherapy (total minimum tumor dose 6485 cGy, maximal dose 6575 cGy). The patient is alive 9.5 years after treatment without evidence of tumor recurrence and with only focal median nerve functional deficits. A review of the patient's treatment is warranted to provide a description of a regimen that may be useful in the treatment of similar patients in the future.