The Authors report a case of a 49 year old female patient with Gardner's Syndrome and adenocarcinoma of the Ampulla of Vater. In this case, adenocarcinoma of the Ampulla of Vater was associated with duodenal polyp (adenoma), which suggests that adenocarcinoma is the consequence of malignant of duodenal polyps. The world medical literature was reviewed and 29 cases of periampullary carcinoma complicating familial polyposis or Gardner's Syndrome were analyzed.

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