Korean J Head Neck Oncol.  2023 Nov;39(2):45-48. 10.21593/kjhno/2023.39.2.45.

A Case of Early Malignant Change from Neurofibroma to Malignant Peripheral Nerve Sheath Tumor

Affiliations
  • 1Department of Otolaryngology, Head and Neck Surgery, Kosin University College of Medicine, Busan, Korean
  • 2Department of Pathology, Department of Pathology, Kosin University College of Medicine, Busan, Korean

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.

Keyword

Neurofibromatosis 1⋅Neurofibroma⋅Malignant peripheral nerve sheath tumor
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