Background : Tumors of the ampulla of Vater are uncommon lesions accounting for only 0.5% of the gastrointestinal tumors. Ampulla of Vater tumors are difficult to apply the prosper resection methods such as endoscopic papillectomy, transduodenal ampullectomy and pancreaticoduodenectomy.

Methods : A Sixty three years old Korean man, who is a non-smoker, hypertensive and diabetic, presented to the hospital with severe epigastric pain. Physical examination and laboratory tests were unremarkable except for a mild elevation of the pancreatic enzyme. Liver enzyme and CA19-9 levels were normal. An abdominal CT scan revealed intrahepatic, extrahepatic hepatic biliary tree and pancreatic duct dilatation with gallbladder stones. He underwent cholecystectomy and transduodenal ampullectomy and both the CBD and pancreatic duct were re-implanted in the duodenum. The pathology was ampullary adenoma with atypia

Results : Endoscopic follow up for ampulla of Vater revealed recurrence of adenoma. Four and half years later, ampullary adenoma changed adenocarcinoma through the high grade dysplasia. He underwent Whipple procedure. Pathology showed Ampulla of Vater carcinoma T3N1M0 stage II B. Despite of chemotherapy and radiation, he had recurrent cancer in the liver and the retroperitoneum 2 years later.

Conclusions : We conclude that proper treatment for recurrent ampulla of Vater adenoma is pylorus preserving pancreaticoduodectomy.