Carcinoma Arising in the Wall of Congenital Bile Duct Cysts

The incidence of carcinoma arising in the wall of the congenital bile duct cysts is much higher than previously assumed. The authors report 4 such cases of primary and secondary carcinomas and review their clinical features through the similar 59 cases in the literature. Of the 63 cases, the average age was much younger, at least several decades, compared with cases of extra-hepatic carcinoma without bile duct cysts. The female-male ratio was 2.5:1. Racial preponderance was also observed, namely, the majority were Japanese. Additionally, many patients previously received various internal drainage procedures, especially choledochocystoduodenostomy. As the treatment, primary excision of the extrahepatic bile duct cyst seems to give the best results because it can avoid ascending cholangitis and prevent development of carcinoma. However, carcinoma still can arise in the intrahepatic bile duct cyst, which cannot be removed at the present time.