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

Cholangiocarcinoma:

• 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)