DOI QR코드

DOI QR Code

제1형 신경섬유종증, 신경섬유종, 악성 말초신경초종

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

  • 강푸름 (고신대학교복음병원 이비인후-두경부외과학교실) ;
  • 김영준 (고신대학교복음병원 이비인후-두경부외과학교실) ;
  • 오선주 (고신대학교복음병원 병리과교실) ;
  • 이형신 (고신대학교복음병원 이비인후-두경부외과학교실)
  • Poo Reum Kang (Department of Otolaryngology, Head and Neck Surgery, Kosin University College of Medicine) ;
  • Yeong Joon Kim (Department of Otolaryngology, Head and Neck Surgery, Kosin University College of Medicine) ;
  • Sun Ju Oh (Department of Pathology, Kosin University College of Medicine) ;
  • Hyoung Shin Lee (Department of Otolaryngology, Head and Neck Surgery, Kosin University College of Medicine)
  • 투고 : 2023.08.21
  • 심사 : 2023.09.27
  • 발행 : 2023.11.30

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

참고문헌

  1. Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W. Prognosis and risk factors for malignant peripheral nerve sheath tumor: A systematic review and meta-analysis. World J Surg Oncol. 2020;18:1-12
  2. Goertz O, Langer S, Uthoff D, Ring A, Stricker I, Tannapfel A, et al. Diagnosis, treatment and survival of 65 patients with malignant peripheral nerve sheath tumors. Anticancer Res. 2014;34:777-783.
  3. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986;57:2006-2021.
  4. Evans DGR, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002;39:311-314.
  5. Im SI. How to write case reports in medicine. Kosin Med. 2022;37:102-106.
  6. Markham A, Keam SJ. Selumetinib: First approval. Drugs. 2020;80:931-937.
  7. Dombi E, Solomon J, Gillespie A, Fox E, Balis F, Patronas N, et al. NF1 plexiform neurofibroma growth rate by volumetric MRI: Relationship to age and body weight. Neurology. 2007;68:643-647.
  8. Ly KI, Merker VL, Cai W, Bredella MA, Muzikansky A, Thalheimer RD, et al. Ten-year follow-up of internal neurofibroma growth behavior in adult patients with neurofibromatosis type 1 using whole-body MRI. Neurology. 2023;100:e661-e670.
  9. Chopra R, Morris CG, Friedman WA, Mendenhall WM. Radiotherapy and radiosurgery for benign neurofibromas. Am J Clin Oncol. 2005;28:317-320.