胃・十二指腸多発腺腫および十二指腸乳頭部癌を契機に発見されたGardner症候群の1例

Gardner's Syndrome Initially Suggested by Gastroduodenal Adenomas and Adenocarcinoma of the Duodenal Papilla, Report of a Case

要旨　患者は45歳の男性．1995年5月，人間ドックで胃隆起性病変を指摘され，当科で上部消化管内視鏡検査を行い，多発性の胃腺腫，十二指腸腺腫および十二指腸乳頭部癌を認めた．家族性大腸腺腫症を疑い大腸内視鏡検査を行ったところ，非密生型の大腸ポリポーシスを認めた．全身の検索で，前頭骨部に骨腫，前胸部に類表皮囊胞を認め，Gardner症候群と診断した．X線，EUS，ERCPで十二指腸乳頭部の腫瘍は粘膜内癌と診断し，外科的ポリペクトミーを行った．腫瘍は3.0×2.3cmの粘膜内にとどまる乳頭状腺癌であった．近年，家族性大腸腺腫症患者において，上部消化管腫瘍性病変の報告が増加しているが，本例も大腸腺腫症患者における上部消化管検査の重要性を裏付ける症例と思われた．

　A 45-year-old man was admitted to our hospital because gastric radiography at a medical ckeckup disclosed abnormal findings. Upper gastrointestinal endoscopy and biopsies showed multiple tubular adenomas in the antrum and duodenum, and a well differentiated adenocarcinoma at the major papilla of the duodenum. Further examinations revealed adenomatosis of the colorectum, osteoma, and the epidermoid cyst of the breast. Therefore, he was diagnosed as having Gardner's syndrome. Because radiographic and endoscopic ultrasonographic findings showed that the carcinoma at the papilla was localized within the mucosal layer, open surgical polypectomy of the tumor was performed. The tumor was intramucosal papillary adenocarcinoma measuring 3.0×2.3 cm in size. Recent reports reveal that multiple neoplastic lesions do exist in the upper gastrointestinal tract in patients with FAP. Thus, careful examinations by upper gastrointestinal endoscopy and radiography should be undertaken to detect the neoplastic lesions while they are in the early stage of growth.