Arch Plast Surg.  2020 Jan;47(1):92-96. 10.5999/aps.2019.01529.

Soft tissue sarcoma in neurofibromatosis type 1: A rare case of malignant peripheral nerve sheath tumor of the skin

• Chung YH
• Jang JW
• Cho JY

• KMID: 2501142
• DOI: http://doi.org/10.5999/aps.2019.01529

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare and often aggressive soft tissue sarcoma originating from the sheaths of peripheral nerves. Approximately 50% of MPNSTs occur in patients with neurofibromatosis (NF). These tumors often present as deep soft tissue lesions, arising from the nerve plexuses of the extremities or from the nerves extending from the trunk. They rarely occur in the skin, especially in patients with NF. Herein, we report our experience with an MPNST of the skin in a patient with NF.

Keyword

Neurofibromatosis 1; Nerve sheath neoplasm; Skin neoplasm