Published (Version of record)JAAD Case Reports. 4(7),(2018) pp. 716-718.
Abstract
Introduction:
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon, aggressive sarcoma that arises from peripheral nerves and metastasizes hematogenously, most often to lung and bone. Approximately half of MPNSTs arise from plexiform neurofibromas in patients with neurofibromatosis type I (NF1). The remaining half occur sporadically, 10% of which arise from previously irradiated tissue. The epithelioid variant of MPNST is uncommon, accounting for less than 5% of cases. The most important aspect of treatment for MPNST is achieving clear surgical margins, as survival decreases dramatically with positive margins. Similar to other soft tissue sarcomas (STS), adjunctive chemoradiotherapy is used if wide margins cannot be achieved, even though MPNST is notoriously resistant to these therapies; the average 5-year survival rate for patients with MPNST is 50% to 60%, with even lower rates for metastatic disease. There are only 2 reports of MPNST metastasizing to the skin, and there are no reports of using intralesional methotrexate to slow progression of cutaneous metastases.