Clinical Characteristics of Biliary Tract Cancer Associated with Choledochal Cyst

ABSTRACT

PURPOSE: Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extra-hepatic bile ducts. The mechanism of carcinogenesis in choledochal cyst has not been clearly elucidated, although stasis of bile and reflux of pancreatic juice appear to be important factors. The aim of this study was to identify the clinical risk factors predicting development of biliary tract cancers in patients with choledochal cyst. METHODS: The study subjects included 170 consecutive patients who underwent surgery for choledochal cysts at Seoul National University Hospital between December 1980 and May 2008. We analyzed the demographic characteristics, clinical symptoms, laboratory findings, type of choledochal cysts, pathologic characteristics, and long-term outcomes of the patients with associated biliary tract cancers. RESULTS: Out Of 170 patients with choledochal cysts, combined biliary tract cancers ware identified in 29 patients, which included extrahepatic bile duct (n=15; 51.7%), gallbladder (n=12; 41.4%), and ampulla of Vater cancers (n=2; 6.9%). There were no significant differences in gender, clinical symptoms (abdominal pain, jaundice, and abdominal masses), laboratory findings (leukocytosis, hyperbilirubinemia, and increased alkaline phosphatase), and Todani classification of choledochal cysts between the two groups with or without combined biliary tract cancer. Multivariate analysis revealed that age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. CONCLUSION: Age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. Therefore, the possibility of associated biliary tract cancers should be considered when planning surgical management for patients with these risk factors.