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Synchronous double cancers in the biliary system are rare. Double cancer of the common bile duct (CBD) and the ampulla of Vater without pancreaticobiliary maljunction (PBM) is extremely rare. To our knowledge, only 7 cases have been reported previously. Here we report a case of synchronous double cancer of the CBD and the ampulla of Vater without PBM. A 63-year-old man was referred to our hospital with epigastric pain and jaundice. Computed tomography (CT) showed dilatation of both intrahepatic and intrapancreatic bile ducts, and slightly enhanced tumor in the middle part of the CBD and the ampulla of Vater. Endoscopic retrograde cholangiopancreatography (ERCP) showed a tuberous filling defect in the middle part of the CBD and an exposed tumor of the ampulla of Vater. Under a diagnosis of synchronous double cancer of the middle bile duct and the ampulla of Vater, pancreaticoduodenectomy was performed. Histopathologically, the ampulla of Vater tumor was well-differentiated tubular adenocarcinoma while the middle bile duct tumor was moderately-differentiated tubular adenocarcinoma. Normal mucosa was interposed between both tumors. These findings suggest that they were 2 primary tumors. Double cancer of the CBD and the ampulla of Vater without PBM is extremely rare. We report a case of synchronous double cancer of the CBD and the ampulla of Vater without PBM.

Synchronous Double Cancer of the Common Bile Duct and the Ampulla of Vater Without Pancreaticobiliary Maljunction: A Case Report