Pancreatobiliary maljunction: association with gallbladder cancer.

BACKGROUND: Pancreatobiliary maljunction is a rare disease characterized by the junction of the pancreatic and biliary ducts outside of the duodenal wall, which normally results in a large common duct. As a result, there is a greater risk of acute pancreatitis and cancer of the gallbladder and biliary tract. CASE REPORT: We present the case of a 43-year-old female diagnosed with a pancreatobiliary maljunction and an associated stenosis of the bile duct, secondary to an episode of acute pancreatitis. She underwent several endoscopic retrograde cholangiopancreatography procedures over the course of three years, without improvement of the stenosis, and therefore a surgical approach was taken. Prior to the surgical intervention, magnetic resonance imaging showed the presence of an 11-mm polyp in the gallbladder. A histological study of the surgical sample identified intramucosal adenocarcinoma over a tubular adenoma of the gallbladder. DISCUSSION: Pancreatobiliary maljunction can be considered as a premalignant entity due to the risk of developing cancer of the biliary tree and gallbladder. Therefore, these patients should undergo a prophylactic intervention, despite being asymptomatic.