Cholangiocarcinoma can present anywhere along the biliary tree. Clinical presentation, preoperative evaluation, and treatment are dependent on the anatomic location of the tumor. Cholangiocarcinomas are divided into those that arise in the extrahepatic and those that arise in the intrahepatic bile ducts. Cholangiocarcinomas originating in the extrahepatic bile ducts are categorized as distal, mid, and proximal (hilar). Distal tumors are those lying in the retroduodenal and intrapancreatic bile duct. Mid bile duct tumors lie below the right and left bile-duct confluence but above the retroduodenal common bile duct. Proximal bile-duct tumors lie at or above the right–left bile-duct confluence.
Patients with extrahepatic cholangiocarcinoma most frequently present with jaundice but have often had more non-specific accompanying symptoms for a few weeks to months prior to the onset of jaundice. Those additional symptoms include pruritus, mild abdominal pain, anorexia, and weight loss. Infrequently, asymptomatic patients with extrahepatic cholangiocarcinoma will be diagnosed because of an unexplained rise in serum alkaline phosphatase or bilirubin or because an imaging study done to evaluate for another condition demonstrates dilated bile ducts. The age at presentation is most frequently 60 years or greater, but younger patients, especially those with ulcerative colitis, primary sclerosing cholangitis, or choledochal cyst, may present with cholangiocarcinoma.
Initial laboratory assessment of patients with jaundice or with dilated bile ducts should include liver functions: total bilirubin, fractionated bilirubin, alkaline phosphatase, aspartate transaminase (AST), alanine transaminase (ALT), serum albumin; complete blood count, including platelet count; prothrombin time and partial thromboplastin time; tumor markers, including carcinoembryonic antigen (CEA), cancer antigen (CA)19–9, and alpha-fetoprotein (AFP); blood urea nitrogen (BUN) and creatinine; and hepatitis serologies, including hepatitis A antibody, hepatitis B surface antigen and antibody, hepatitis B core antibodies, and hepatitis C antibody.