A cholangiocarcinoma occurring at the confluence of the right and left hepatic bile ducts.
Cholangiocarcinoma accounts for approximately 2% of all cancer diagnoses, with an overall incidence of 1.2/100,000 individuals.
Two-thirds of cases over the age of 65, with a near ten-fold increase in patients over 80 years of age.
The incidence is similar in both men and women.
Accounts for 10% of liver and biliary tumors.
Become symptomatic late in their development and therefore are not usually resectable.
Primary sclerosing cholangitis, an idiopathic inflammatory condition of the biliary tree, is associated with the development of cholangiocarcinoma in up to 40% of patients.
Congenital biliary cystic disease, is associated with malignant transformation in up to 25% of cases, with an anomalous pancreatico-biliary duct junction and reflux of pancreatic secretions into the bile duct.
Chronic biliary tract parasitic infection due to Clonorchis sinensis and Opisthorchis viv2242ini, have also been identified as a risk factors.
Exposure to asbestos and nitrosamines, the radiologic contrast agent, Thorotrast (thorium dioxide), are considered to be risk factors.