Approach to the diagnosis of cholangiocarcinoma in a patient who does not have primary sclerosing cholangitis (PSC)

CA 19-9: carbohydrate antigen 19-9; CEA: carcinoembryonic antigen; AFP: alpha-fetoprotein; US: ultrasound; CT: computed tomography; MRI: magnetic resonance imaging; MRCP: magnetic resonance cholangiopancreatography; MDCT: contrast-enhanced multiphasic multidetector row computed tomography; CCA: cholangiocarcinoma; EUS: endoscopic ultrasound; FNA: fine needle aspiration; ERCP: endoscopic retrograde cholangiopancreatography; PET: positron emission tomography; IDUS: intraductal ultrasound; PTC: percutaneous transhepatic cholangiography.
* A diagnosis of CCA should be considered if there are signs of biliary obstruction (eg, jaundice, abnormal liver tests in a cholestatic pattern, bile duct dilation on imaging studies) without an alternative explanation (eg, choledocholithiasis, a pancreatic head lesion). The diagnosis should also be considered in patients with an isolated intrahepatic mass on imaging and a normal serum level of AFP.
¶ All patients with suspected CCA should have tumor markers (CA 19-9, CEA, and for patients with intrahepatic lesions, AFP) checked. Elevated tumor markers may support a diagnosis of CCA or, in the case of an elevated AFP, suggest an alternative diagnosis (hepatocellular carcinoma). CA 19-9 is elevated if it is >37 units/mL.
Δ If at any point during the evaluation an alternative diagnosis is made, treatment should be instituted as appropriate for that diagnosis.
◊ If the patient is a surgical candidate. Preoperative PET/CT scan is of utility primarily for identifying occult metastases.
§ EUS is preferred in many centers for patients with extrahepatic bile duct dilation unless drainage is required or tissue cannot be obtained with FNA. For patients with a perihilar mass or isolated intrahepatic bile duct dilation, ERCP is the next test obtained since the lesion may not be visualized on EUS.
¥ With brushings/biopsies of any intraductal lesions or strictures. Obstructing lesions can be stented if needed. If ERCP is nondiagnostic, consider cholangioscopy (if available) for direct visualization of the bile duct and biopsies. IDUS (if available) can be used to further define the extent of the tumor. If the lesion is not accessible endoscopically, consider PTC.