Extrahepatic cholangiocarcinoma: clinical features

Extrahepatic cholangiocarcinomas (E-CCAs), are hepatobiliary cancers with features of cholangiocyte differentiations, originating from extrahepatic biliary tree at the bifurcation of the hepatic ducts and also in the distal duct. E-CCAs represent the most common type of cholangiocarcinomas (CCAs) and are characterized by poor overall survival. The principal risk factors for E-CCAs are strictly related to geographic location. Jaundice is the most common physical sign at disease presentation. Other common more non-specific symptoms include hepatomegaly, right upper qudrant mass, weight loss, vomiting, nausea, diarrhoea, malaise and fatigue. Unlike intrahepatic CCA, an incidental asymptomatic presentation occurs in a small percentage of cases. This chapter evaluates the principal clinical features of E-CCAs, briefly discussing its specific risk factors.