ABSTRACT
The vast majority of ampullary tumors are epithelial in nature and are either adenoma or adenocarcinoma. Rarely lymphoma or mucinous and squamous elements may be found. Primary lymphoma of pancreaticobiliary region are exceedingly rare, and mostly nodal. Recently, we have experienced a 29-year-old woman with primary malignant lymphoma of the ampulla of Vater with recurrent acute pancreatitis and jaundice. On duodenoscopy, bulging ampulla with normal overlying duodenal mucosa was observed. Endoscopic sphincterotomy was done and tumor inside the ampulla was exposed. ERCP showed high grade biliary and pancreatic ductal strictures extending from the papillary orifice, with upstream ductal dilatation, respectively. With endoscopic biopsy alone, the tumor was confirmed as B-cell lymphoma histologically.