von Recklinghausen 病に合併した下顎歯肉癌の1例 A case of squamous cell carcinoma of the lower gingiva complicated by von Recklinghausen's disease

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Neurofibromatosis (NF) type 1, known as von Recklinghausen's disease, is an autosomal dominant inherited disorder caused by a mutation of a gene on the long arm of chromosome 17. Malignant tumors are often observed in patients with NF, whereas oral carcinoma concurrent with NF is very rare. This paper describes a case of squamous cell carcinoma of the lower gingiva adjacent to a neurofibroma in a patient with NF type 1.<br>A 57-year-old man with NF type 1 was referred to our hospital. Intraoral examination revealed two masses: one was a firm nodule on the buccal shelf of the lower left posterior region covered by ulcerated mucosa, and the other was a soft to firm nodule adjacent to the other one in the left cheek region and was covered by normal mucosa.<br>Excisional biopsy of the two nodules was done. Histological examination showed that the nodule in the buccal region was squamous cell carcinoma (T2N0M0), and the other was a usual type of neurofibroma. The patient underwent chemotherapy and radiotherapy, followed by local resection of the carcinoma. Local recurrence developed 1 month later. Mandibular segmental resection including the region of neurofibroma, followed by neck dissection and reconstruction with a rectus abdominus free flap was done. The patient has not had tumor recurrnce or metastasis for 8 years after mandibular resection.

Neurofibromatosis (NF) type 1, known as von Recklinghausen's disease, is an autosomal dominant inherited disorder caused by a mutation of a gene on the long arm of chromosome 17. Malignant tumors are often observed in patients with NF, whereas oral carcinoma concurrent with NF is very rare. This paper describes a case of squamous cell carcinoma of the lower gingiva adjacent to a neurofibroma in a patient with NF type 1.<br>A 57-year-old man with NF type 1 was referred to our hospital. Intraoral examination revealed two masses: one was a firm nodule on the buccal shelf of the lower left posterior region covered by ulcerated mucosa, and the other was a soft to firm nodule adjacent to the other one in the left cheek region and was covered by normal mucosa.<br>Excisional biopsy of the two nodules was done. Histological examination showed that the nodule in the buccal region was squamous cell carcinoma (T2N0M0), and the other was a usual type of neurofibroma. The patient underwent chemotherapy and radiotherapy, followed by local resection of the carcinoma. Local recurrence developed 1 month later. Mandibular segmental resection including the region of neurofibroma, followed by neck dissection and reconstruction with a rectus abdominus free flap was done. The patient has not had tumor recurrnce or metastasis for 8 years after mandibular resection.

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  • 日本口腔外科学会雑誌

    日本口腔外科学会雑誌 57(5), 309-313, 2011-05-20

    Japanese Society of Oral and Maxillofacial Surgeons

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各種コード

  • NII論文ID(NAID)
    10030272130
  • NII書誌ID(NCID)
    AN00189163
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    00215163
  • NDL 記事登録ID
    11132045
  • NDL 雑誌分類
    ZS44(科学技術--医学--歯科学・口腔外科学)
  • NDL 請求記号
    Z19-145
  • データ提供元
    CJP書誌  NDL  J-STAGE 
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