Gardner症候群患者の上顎にみられた明細胞性歯原性癌の1例

  • 土井 理恵子
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野
  • 小谷 勇
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野
  • 木谷 憲典
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野
  • 中林 基
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野
  • 岡本 秀治
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野
  • 領家 和男
    鳥取大学医学部感覚運動医学講座口腔顎顔面病態外科学分野

書誌事項

タイトル別名
  • A case of clear cell odontogenic carcinoma arising in the upper jaw of a patient with Gardner syndrome
  • Gardner ショウコウグン カンジャ ノ ジョウガク ニ ミラレタ アキラ サイボウセイシ ゲンセイガン ノ 1レイ

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Clear cell odontogenic carcinoma (CCOC) was reclassified from a benign tumor to a malignant tumor by the WHO classification in 2005 because of its aggressive nature and potential for local recurrence and distant metastases. It is a rare epithelial tumor of odontogenic origin. Only 82 well-documented cases have been reported to date to the best of our knowledge. Gardner syndrome is familial adenomatous polyposis (FAP), characterized by osteoma, soft tissue tumor, and colorectal polyposis. We report a case of CCOC arising in the upper jaw of a patient with with Gardner syndrome.<br>A 34-year-old woman with Gardner syndrome was referred to our clinic because of rapid swelling of the left side of the maxilla. Malignancy was suspected on biopsy. She had a history of two operations at the same region. Computed tomography showed a bone defect in the upper jaw, with invasion to the surrounding soft tissue. We diagnosed CCOC of the left side of the upper jaw and performed tumor resection and prophylactic functional neck dissection. There was no recurrence or metastasis after a follow-up period of 4 years 8 months. Histologically, the tumor showed a biphasic pattern characterized by cords and nests of clear cells scattered with islands of cells with eosinophilic cytoplasm. We finally diagnosed CCOC. We discuss this rare case of CCOC with Gardner syndrome and review the literature.

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