Metachronous cholangiocarcinoma 13 years post resection of choledochal cyst-is long-term follow-up useful?: a case study and review of the literature.
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.


METHODS
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.
Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
Deanna Wan Jie
DW
Ng
Hepatopancreatobiliary Unit, Department of General Surgery, Changi General Hospital, Simei Street 3, Singapore, 529889, Singapore. adrian_chiow@cgh.com.sg.
Adrian Kah Heng
AK
Chiow
Department of Pathology, Changi General Hospital, Singapore, Singapore.
Wee Teng
WT
Poh
Hepatopancreatobiliary Unit, Department of General Surgery, Changi General Hospital, Simei Street 3, Singapore, 529889, Singapore.
Siong San
SS
Tan
Cholangiocarcinoma; Choledochal cyst; Metachronous biliary tract cancer