Introductory Chapter: Bile Duct Cancer

Cholangiocarcinoma (CC) is the most characteristic malignant tumour affecting to the bile ducts. It is a rare neoplasm that originates in the cells of the biliary epithelium. In the USA, it has a mean incidence of 1 case/100,000 inhabitants/year. They are classified into three groups, with respect to their location within the biliary tree: (1) intrahepatic, (2) the upper and middle thirds and (3) the distal part of the common bile duct. Intrahepatic tumours are the least frequent. They behave like primary hepatic tumours. Perihilar, or Klatskin, tumours are the most frequent, accounting for 60–80% of the total. Their incidence increases with age, more cases appearing in patients aged between 50 and 70 years, with a clear predominance in men. The diseases most frequently associated with it are primary sclerosing cholangitis (PSC), choledochal cysts, chronic infections with the parasite Clonorchis sinensis (typical of Asian countries and with a predominant intrahepatic location) and the presence of cholelithiasis and choledocholithiasis. The presence of PSC is an important risk factor, given that it occurs in 30% of cases. In many patients, no known risk factors are found.