Choledochal cyst cholangiocarcinoma arising from adenoma: case report and a review of the literature.

A case of cholangiocarcinoma arising in an unresected choledochal cyst in adulthood is presented. Although typically diagnosed in pediatric population, as many as 20% to 30% of choledochal cysts can be discovered in adulthood. Unresected choledochal cyst is clearly associated with increased risk of cholangiocarcinoma. Proper surgical treatment includes cyst resection and bilioenteric anastomosis. Asymptomatic patients with choledochal cyst previously treated by biliary diversion without cyst resection present a challenging issue and should be considered for cyst resection. Association of choledochal cyst with pancreaticobiliary anomalies is reviewed.