제1형 신경섬유종증, 신경섬유종, 악성 말초신경초종

• 영문명: A Case of Early Malignant Change from Neurofibroma to Malignant Peripheral Nerve Sheath Tumor
• 발행기관: 대한두경부종양학회
• 저자명: 강푸름 김영준 오선주 이형신
• 간행물 정보: 『대한두경부종양학회지』제39권 제2호, 45~48쪽, 전체 4쪽
• 주제분류: 의약학 > 종양학
• 파일형태: PDF
• 발행일자: 2023.11.30

국문 초록

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.

키워드

Neurofibromatosis 1 Neurofibroma Malignant peripheral nerve sheath tumor