The goal of diagnostic staging of cholangiocarcinoma is to evaluate resectability and extent of surgery, because surgery is the only curative therapy. Cure and long-term survival can be achieved if the tumor is completely excised and the histologic margins are negative.

Staging of mass-forming intrahepatic cholangiocarcinoma is performed using contrast-enhanced MRI or CT. The extent and number of tumor nodules, associated portal vein narrowing or occlusion, and the presence of lymph node and distant metastasis are important determinants of survival after surgery.

Extrahepatic cholangiocarcinoma is more challenging and may require more than one imaging modality for staging. Extrahepatic tumors are classified according to their anatomical location into perihilar (Klatskin) and distal tumors. Perihilar cholangiocarcinoma is typically located in the extrahepatic duct proximal to the insertion of the cystic duct. Distal tumors arise anywhere from below the confluence of the cystic duct with the common bile duct to the ampulla of Vater. In this chapter, we focus on the staging and preoperative evaluation of extrahepatic cholangiocarcinoma.

Perihilar extrahepatic cholangiocarcinoma

The preoperative evaluation of perihilar cholangiocarcinoma must address four critical determinants of resectability:

1. Extent of tumor within the biliary tree,

2. Vascular invasion,

3. Hepatic lobar atrophy, and

4. Metastatic disease

In the absence of metastatic disease, the location and extent of tumor spread along the bile ducts will determine whether it can be completely excised.

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.

Preoperative assessment

Extrahepatic cholangiocarcinoma

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.