Abstract

Pancreaticobiliary maljunction (PBM) and congenital biliary dilatation (CBD) are important risk factors of biliary cancers. According to the nationwide survey in Japan, the biliary cancer incidence of PBM was 21.6% in patients with CBD and 42.4% in patients without CBD. In patients with PBM and CBD, resection and reconstruction of the extrahepatic bile duct (flow-diversion surgery) is a basic surgical procedure for preventing the high occurrence of bile duct and gallbladder cancer. On the contrary, in patients with PBM but without CBD, no consensus has been reached about whether it is better to perform cholecystectomy alone or flow-diversion surgery. After surgery, a reported 0.5–2.0% of patients who undergo cyst excision develop bile duct carcinoma. Although the incidence of cancer after surgery is lower than that before surgery, it is 120–200 times higher than that of the entire population. Therefore, long-term follow-up is needed in patients with PBM and/or CBD.

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