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Papillary carcinoma is a rare tumor of the gallbladder. Papillary mucinous lesions of the intra- and extra-hepatic biliary tract (BT- IPMN) have been recognized. However the gallbladder is not included, except for the diffuse papillomatosis, where the sequence biliary papillomatosis to papillary carcinoma is proposed. We report a simultaneous case of gallbladder papillary neoplasia and intrahepatic duct carcinoma in situ associated with pancreaticobiliary maljunction (PBM). We proposed that double location, in our case, is more likely explained by a diffuse biliopancreatic tree disease leading to synchronous tumors arising in amenable duct. It was verified absence of continuity between gallbladder and intrahepatic bile duct site of involvement, absence of lymph node metastasis or venous involvement. This case report supports the concept of a proliferative and neoplastic process involving simultaneously the biliary tree and gallbladder associated with PBM.

Gallbladder Papillary Neoplasia Associated With Intrahepatic Carcinoma and Pancreaticobiliary Malformation