Intrahepatic cholangiocarcinoma arising 10 years after the excision of congenital extrahepatic biliary dilation

Abstract

A 52-year-old woman was found to have a liver tumor during treatment for a liver abscess. The tumor was diagnosed as intrahepatic cholangiocarcinoma by closer examinations, including a percutaneous needle biopsy. Ten years previously, she had undergone excision of a choledochal cyst, with reconstruction by Roux-en-Y hepaticojejunostomy, as treatment for Todani's type Ia congenital biliary dilation, which had been confined only to the extrahepatic bile duct. The significant association between congenital biliary dilation and hepatobiliary malignancies is well known. Some patients have been reported to develop biliary cancer long after the excision of the entire extrahepatic bile duct and hepaticoenterostomy. However, in these patients, the development mostly took place in the remnant choledochal cyst, the anastomotic site, or in the dilated intrahepatic bile duct of Todani's type IV-A congenital biliary dilation. The development of intrahepatic cholangiocarcinoma after operation has not been reported previously in a patient with Todani's type I congenital biliary dilation. This case suggests that the entire biliary tree may have a high risk of field cancerization, even in extrahepatic congenital biliary dilation.

Cholangiocarcinoma: Modern advances in understanding a deadly old disease

“…A likely distinct type of cholangiocarcinoma arises in the setting of fibropolycystic malformations of the biliary tree; indeed there is a 1% per year cumulative increase in cancer risk in patients with choledochal cysts. While biliary obstruction and inflammation play a role, other factors increase the risk of cancer, as cholangiocarcinomas can occur decades after surgical resection of the cyst (14). One unifying feature of all these risk factors for cholangiocarcinoma is the presence of chronic biliary inflammation.…”

Choledochal Cyst: A Report of 3 Cases and a Review of its Treatment and Outcome

“…There is immediate relief of symptoms with either of these technique, but the risk of complications and malignancy in the residual cyst can be high, as high as 2.5-26% [50,51]. Anastomotic site stricture, formation of bile stones, tendency for recurrent cholangitis due to bile stasis are commonly associated internal drainage procedures [52].…”

Intrahepatic Cholangiocarcinoma Arising 33 Years After Excision of a Choledochal Cyst: Report of a Case

“…[1][2][3][4] The regurgitation of pancreatic juice into the bile duct and the accumulation of bile juice in an anomalous pancreatobiliary ductal junction leads to chronic changes and may stimulate genetic alterations in patients with an anomalous pancreatobiliary ductal junction. 1,[5][6][7][8] Biliary tract carcinoma rarely arises after biliary diversion surgery, such as roux-en-Y hepaticojejunostomy, but the remaining bile duct may predispose to carcinoma development for several years afterward. 1,5 Cases of cancer development in the biliary system more than 10 years after diversion surgery have been reported.…”

“…1,[5][6][7][8] Biliary tract carcinoma rarely arises after biliary diversion surgery, such as roux-en-Y hepaticojejunostomy, but the remaining bile duct may predispose to carcinoma development for several years afterward. 1,5 Cases of cancer development in the biliary system more than 10 years after diversion surgery have been reported. 1,5,[9][10][11][12][13] However, it is unknown whether such patients had carcinogenic potential for several decades after diversion surgery.…”

“…1,5 Cases of cancer development in the biliary system more than 10 years after diversion surgery have been reported. 1,5,[9][10][11][12][13] However, it is unknown whether such patients had carcinogenic potential for several decades after diversion surgery. Cancer could develop postoperatively due to cholestasis or it could develop de novo in patients after diversion surgery, as can occur in healthy individuals.…”