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

  • Chung, Young Hun (Department of Plastic Surgery, Kyung Hee University Graduate School) ;
  • Jang, Jin Woo (Department of Plastic Surgery, Kyung Hee University Medical Center) ;
  • Cho, Jae Young (Department of Plastic Surgery, Kyung Hee University Medical Center)
  • Received : 2019.10.16
  • Accepted : 2019.11.06
  • Published : 2020.01.15

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.

Keywords

References

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