Abstract
BACKGROUND: Patients with congenital choledochal cyst are at risk of the development of hepatobiliary malignancy, with recommended treatment of choledochal cyst being surgical excision. The development of cholangiocarcinoma more than 10 years after excision of choledochal cysts is rare, with less than 21 cases reported in the literature from 1972 to 2014. This is the first reported case of metachronous recurrence after a previously excised adenocarcinoma within a choledochal cyst. CASE PRESENTATION: Herein, we review the case of a patient with cholangiocarcinoma arising 13 years post excision of a Todani type 1 choledochal cyst and discuss the theories of carcinogenesis and long-term management of patients with choledochal cysts. The long-term development of a malignancy must be considered in these patients. CONCLUSIONS: Reviewing all published cases to date, regular follow-up post resection did not improve on the resectability and long-term survival of these patients. Patients presenting with symptoms did not prejudice against resectability. Despite curative resection, median survival was dismal. Optimal long-term follow-up strategies for these patients remain to be elucidated.