Approach to the diagnosis of cholangiocarcinoma in a patient with primary sclerosing cholangitis

PSC: primary sclerosing cholangitis; CA 19-9: carbohydrate antigen 19-9; CEA: carcinoembryonic antigen; AFP: alpha-fetoprotein; MRCP: magnetic resonance cholangiopancreatography; CCA: cholangiocarcinoma; ERCP: endoscopic retrograde cholangiopancreatography; PET: positron emission tomography; CT: computed tomography; IDUS: intraductal ultrasound; PTC: percutaneous transhepatic cholangiography.
* In patients with PSC, CCA should be considered if there is rapid clinical deterioration with jaundice, weight loss, and abdominal pain. The presence of progressive biliary dilatation in the setting of a dominant stricture or thickening of the bile duct wall should also raise a strong suspicion of CCA. CCA should also be considered if suggested by screening test results (ie, CA 19-9 and/or MRCP).
¶ All patients suspected of having 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). In patients with PSC, the optimal CA 19-9 cutoff (providing optimal balance between sensitivity and specificity) is approximately 100 to 129 units/mL.
Δ If at any point during the evaluation, an alternative diagnosis is made, treatment should be instituted as appropriate for that diagnosis.
◊ 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.
§ If the diagnosis of CCA is strongly suspected but imaging has been nondiagnostic. Preoperative PET/CT scan may also help guide surgery by identifying areas more likely to harbor malignancy.
¥ If the patient is a surgical candidate.