Carcinoma arising in the wall of congenital bile duct cysts

Abstract

The incidence of carcinoma arising in the wall of the congenital bile duct cysts is much higher than previously assumed. The authors report 4 such cases of primary and secondary carcinomas and review their clinical features through the similar 59 cases in the literature. Of the 63 cases, the average age was much younger, at least several decades, compared with cases of extra-hepatic carcinoma without bile duct cysts. The female-male ratio was 2.5:1. Racial preponderance was also observed, namely, the majority were Japanese. Additionally, many patients previously received various internal drainage procedures, especially choledochocystoduodenostomy. As the treatment, primary excision of the extrahepatic bile duct cyst seems to give the best results because it can avoid ascending cholangitis and prevent development of carcinoma. However, carcinoma still can arise in the intrahepatic bile duct cyst, which cannot be removed at the present time.

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

“…[1][2][3][4] Although the underlying mechanism of carcinogenesis in such cases has not been fully elucidated, it is presumed to be the reflux of pancreatic juice into the bile duct and the accumulation of mixed bile in the biliary system caused by an anomalous junction of the pancreaticobiliary duct. To prevent the development of biliary carcinoma, the entire extrahepatic bile duct is excised, and hepaticoenterostomy has been recommended because it separates bile from pancreatic juice flow.…”

“…[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.…”

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

“…Cholangitis, pancreatitis, choledolithias, pancreatic calculi, and sometimes metaplastic changes are common complications of choledochal cyst. Especially cholangitis is commonly associated with types I and IV choledochal cyst because of recurrent reflux of pancreatic juice in the presence of APBDJ [36,37]. Types I and IV choledochal cyst are also the most frequently seen, thus report about its incidence of complications are frequently documented.…”

Estudo comparativo entre a colangiopancreatografia por ressonância magnética e a colangiopancreatografia endoscópica no diagnóstico das obstruções biliopancreáticas

“…Concluíram que formas precoces de pancreatite crônica podem ser diagnosticadas por este método. Um paciente desta casuística apresentava cisto congênito de colédoco, tipo I, na classificação de TODANI et al (30) . A CPRM diagnosticou corretamente a lesão.…”