Cholangiocarcinoma – 7/11/18

Eric presented the case of an elderly woman with no known medical history presenting with subacute onset of painless jaundice and liver failure, found to have perihilar cholangiocarcinoma.

Clinical Pearls:

  • Cholangiocarcinomas are the second most common primary malignancy of the liver after HCC.
  • Perihilar disease is most common.  Can also present with intrahepatic or distal duct involvement.
  • Risk factors include Primary Sclerosing Cholangitis, parasites, and biliary cysts.
  • Metastases occur early in the disease course with the liver being the most common site.
  • Treatment
    • Distal cholangiocarcinoma has the highest resectability.
    • Surgery is the only cure but only a minority of patients present early enough



  • Second most common primary malignancy of liver after HCC
  • Can be intrahepatic, perihilar (most common), or distal
  • Risk factors include:
    • PSC
    • Parasitic infections (liver flukes: clonorchis and Opisthorchis)
    • Biliary atresia
    • Biliary cysts
    • Cholelithiasis, cholecystitis, and hepatolithiasis
  • Mets occur early in disease course except for distal disease
    • Perihilar disease: liver is the most common site of met
    • Intrahepatic: peritoneum, lungs, pleura
    • Distal cholangiocarcinoma: liver, lungs, peritoneum
  • Treatment:
    • Distal disease has the highest resectability
    • Surgery is the only cure but only a minority of patients present early enough
    • Liver transplant in an option for those with
      • perihilar disease
      • < 3 cm tumor size
      • No extrahepatic spread
      • No percutaneous biopsy (increases risk of hematogenous spread)