Abstract
A 47 years old female with familial adenomatous polyposis, who underwent extended left side hemicolectomy for carcinoma of rectosigmoid colon in 1980 and residual total colectomy and rectal mucosectomy in 1985, was found to have a ampullary carcinoma of duodenum. Subsequently pancreatoduodenectomy was performed in 1994. The resected specimens showed an ulcerating carcinoma on the ampulla of Vater, which was a moderately differentiated adenocarcinoma with adenomatous component histologically. Multiple adenomas were present in duodenum and one of them was revealed to be a cancer in adenoma. These observations support the existence of the adenoma-carcinoma sequence in the duodenal lesion of the familial adenomatous polyposis.