Korean Journal of Head & Neck Oncology (대한두경부종양학회지)

The Korean Society for Head and Neck Oncology (대한두경부종양학회)
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Bilateral Occipital Neuralgia in a Patient with Neurofibromatosis Type 1: A Case Report

신경 섬유종증 1형 환자의 양측 후두 신경통: 증례보고

  • Kim, Ji-Young (Department of Plastic and Reconstructive Surgery, Seoul National University College of Medicine) ;
  • Jeon, Sungmi (Department of Plastic and Reconstructive Surgery, Seoul National University College of Medicine) ;
  • Kim, Sang Wha (Department of Plastic and Reconstructive Surgery, Seoul National University College of Medicine)
  • 김지영 (서울대학교 의과대학 성형외과학 교실) ;
  • 전성미 (서울대학교 의과대학 성형외과학 교실) ;
  • 김상화 (서울대학교 의과대학 성형외과학 교실)
  • Received : 2021.07.11
  • Accepted : 2021.09.13
  • Published : 2021.11.30
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Abstract

Plexiform neurofibromas (PNFs) represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1), in which neurofibromas arise from multiple nerves as bulging and deforming masses involving connective tissue and skin folds. We report the case of a 17-year-old man with known NF-1 presenting with bilateral occipital neuralgia that began in his late adolescence. His chief complaint was radiating pain in the occiput induced by protective helmet wear when riding alpine skiing. Craniofacial magnetic resonance imaging (MRI) confirmed the presence of fusiform masses arising from the bilateral greater occipital nerves. Histopathological examination of the biopsy samples showed PNFs. After surgical treatment, the patient's symptoms completely improved. Unlike cutaneous neurofibromas, PNFs have different clinical characteristics and have the risk of malignant mutations. Correct diagnosis and adequate surgical treatment are necessary for PNFs.

Keywords

Acknowledgement

The study was approved by the Institutional Review Board of Seoul National University Hospital (IRB No. H-2106-104-1227) and performed in accordance with the principles of the Declaration of Helsinki. Written informed consent was obtained.

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