Abstract
Background and AimThe aim of this study was to elucidate the risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts.MethodsA retrospective analysis of 94 patients who had undergone cyst excision for congenital choledochal cysts was conducted. The median age at the time of cyst excision and median follow-up time after cyst excision were 7 years and 181 months, respectively.ResultsBiliary tract cancer developed in four patients at 13, 15, 23, and 32 years after cyst excision. The cumulative incidences of biliary tract cancer at 15, 20, and 25 years after cyst excision were 1.6%, 3.9%, and 11.3%, respectively. The sites of biliary tract cancer were the intrahepatic (n = 2), hilar (n = 1), and intrapancreatic (n = 1) bile ducts. Of the four patients with biliary tract cancer after cyst excision, three patients underwent surgical resection and one patient received chemo-radiotherapy. The overall cumulative survival rates after treatment in the four patients with biliary tract cancer were 50% at 2 years and 25% at 3 years, with a median survival time of 15 months.ConclusionsThe risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts seems to be relatively high in the long-term. The risk of biliary malignancy in the remnant bile duct increases more than 15 years after cyst excision. Despite an aggressive treatment approach for this condition, subsequent biliary malignancy following cyst excision for congenital choledochal cysts shows an unfavorable outcome.
Highlights
A condition that predisposes to extrahepatic cholangiocarcinoma is congenital cystic dilatation or choledochal cyst.[1,2,3] these cysts are relatively rare, coexisting carcinoma as a complication has been well documented.[4]
A mixture of bile and pancreatic secretions may promote the development of carcinoma because 90% of choledochal cysts are associated with anomalous pancreaticobiliary ductal junction (APBDJ).[5]
Biliary-enteric anastomosis at larger caliber duct is recommended for prevention of postoperative biliary strictures and subsequent biliary malignancy following cyst excision.[34]
Summary
A condition that predisposes to extrahepatic cholangiocarcinoma is congenital cystic dilatation or choledochal cyst.[1,2,3] these cysts are relatively rare, coexisting carcinoma as a complication has been well documented.[4]. The aim of this study was to elucidate the risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. Results: Biliary tract cancer developed in four patients at 13, 15, 23, and 32 years after cyst excision. The cumulative incidences of biliary tract cancer at 15, 20, and 25 years after cyst excision were 1.6%, 3.9%, and 11.3%, respectively. Conclusions: The risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts seems to be relatively high in the long-term. The risk of biliary malignancy in the remnant bile duct increases more than 15 years after cyst excision. Despite an aggressive treatment approach for this condition, subsequent biliary malignancy following cyst excision for congenital choledochal cysts shows an unfavorable outcome
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