A case of malignant peripheral nerve sheath tumor of the cervical sympathetic nerve associated with neurofibromatosis type 1

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  • Yamamoto Takaya
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Suzuki Kensuke
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Morita Mizuki
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Kawasaki Hiroto
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Sakagami Tomohumi
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Yagi Masao
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University
  • Noda Yuri
    Department of Pathology, Kansai Medical University
  • Iwai Hiroshi
    Department of Otolaryngology, Head and Neck Surgery, Kansai Medical University

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  • 神経線維腫症1型に発症した頸部交感神経由来の悪性末梢神経鞘腫瘍の1例

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Abstract

Neurofibromatosis type 1 （NF-1） is an autosomal dominant inherited disease, in which approximately 3-5％ of patients develop malignant peripheral nerve sheath tumor （MPNST）. In this report, we present a case of MPNST associated with NF-1. The patient was a 26-year-old man presenting with a lump on his right neck. Based on his café-au-lait spots and family history, neurofibroma associated with NF-1 was suspected. The tumor showed rapid growth and pain, and so the patient underwent surgical resection. The final histopathological diagnosis was MPNST. The surgical margin was close, and postoperative radiation therapy was administered. MPNST is a disease with a poor prognosis due to frequent local recurrence and distant metastasis. Although surgical resection for MPNST carries the risk of neurological deficit, early diagnosis and treatment including surgery are important.

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