Acute pancreatitis as the initial manifestation of an adenocarcinoma of the major duodenal papilla in a patient with familial adenomatous polyposis syndrome: a case report and literature review.

We report a case of an ampullary carcinoma presenting as acute pancreatitis in a patient with familial adenomatous polyposis (FAP) syndrome and severe duodenal adenomatosis. A 48-year-old woman was hospitalised because of an episode of acute pancreatitis. She had a history of prophylactic total colectomy for FAP 2 years earlier. On admission, abdominal ultrasonography and computed tomography revealed dilatation of the main pancreatic and common bile duct. Spigelman's stage IV duodenal adenomatosis involving the major duodenal papilla was diagnosed on endoscopy and a classical Whipple procedure was proposed. Pathologic examination of the duodenopancreatectomy specimen revealed a tubular adenocarcinoma of the papilla that occluded the major pancreatic ducts. The patient had no evidence of disease and experienced no recurrent attacks of acute pancreatitis during a 36-month period of follow-up.