家族性大腸腺腫症の術後4年目に発見された十二指腸乳頭部癌の1例

Cancer of the Papilla of Vater Associated with Familial Polyposis Coli, Report of a Case

要旨　患者は37歳の男性で1979年家族性大腸腺腫症，結腸癌と診断され結腸全摘術が施行された．術前の上部消化管検査では胃ポリポージスと十二指腸ポリープが認められたが悪性病変はなかった．家族歴は患者の母親とその姉が直腸癌で死亡しており，また患者の従姉妹が家族性大腸腺腫症，早期結腸癌で同様に手術を受けている．1983年1月，患者が41歳のとき，定期的上部消化管内視鏡検査を受けた．この時点では自・他覚症状，異常検査所見は認められていなかった．この内視鏡検査により十二指腸乳頭部に強く悪性化を示唆する病変が発見された．その部の生検は高分化型腺癌を示した．1983年3月膵頭十二指腸切除術が施行された．切除標本では2cmの乳頭部癌と29個の十二指腸の腺腫性ポリープが認められた．所属リンパ節に転移はなく，癌病巣の膵および胆管への浸潤はなかった．

　In 1979, a 37 year-old man was diagnosed as familial polyposis coli with the colon cancer, and underwent a total colectomy. Preoperative upper gastrointestinal series revealed polyposis of the stomach and duodenal polyps without malignant lesion. His family history showed that his mother and her sister had died of rectal cancer and his cousin had been also operated on for familial polyposis coli with early colon cancer (Fig. 1).

　In January 1983, at the age 41, he underwent periodic gastroduodenal endoscopic examination without signs and symptoms and without abnormal laboratory data. The examination revealed the lesion of the papilla of Vater, which suggested strongly malignant change. Biopsy of the lesion showed well-differentiated adenocarcinoma. In March 1983, the pancreaticoduodenectomy was performed. The surgical specimen contained 29 adenomatous polyps in the second part of the duodenum and a mass in the ampulla of Vater with 2 cm in diameter. There was no metastasis in the resected surrounding lymph nodes. The lesion in the ampulla did not infiltrated pancreas nor bile duct.

　World literature was reviewed and 13 cases of carcinoma originated in the papilla (or ampulla) of Vater associated with familial polyposis coli were summarized (Table 1). Our case was very valuable, because a cancer was detected before some signs and symptoms or abnormal laboratory data appeared. It should be emphasized that the periodic examinations for the upper gastrointestinal tract are essential for familial polyposis coli.