Updated: 7/28/2019

Cholangiocarcinoma

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Snapshot
  • A 35-year-old man with a history of primary sclerosing cholangitis presents with two weeks of jaundice, pruritus, and abdominal pain. He also has noticed that his urine is dark and stools are clay-colored. On physical exam, he has generalized jaundice and a palpable right upper quadrant mass. Lab studies are remarkable for elevated alkaline phosphatase, total bilirubin, alanine aminotransferase, aspartate aminotransferase, and CA 19-9. Magnetic resonance cholangiopancreatography reveals a contrast-enhancing intrahepatic lesion with rim enhancement.  
Introduction
  • Most common malignancy of bile duct
  • Caused by
    • primary sclerosing cholangitis (MCC)
    • thorotrast
    • choledochal cyst
    • Clonorchis sinensis (Chinese liver fluke) 
  • May be located
    • intrahepatically
    • at the junction of R/L hepatic ducts
    • at the ampulla
Presentation
  • Symptoms
    • jaundice
    • pruritus
    • abdominal pain
    • clay-colored stool 
    • dark urine
    • weight loss
    • fever
  • Physical exam
    • palpable gallbladder
      • Courvoisier's sign
    • hepatomegaly
Evaluation
  • Labs
    • tumor markers (AFP, CA 19-9, and CEA) 
  • Imaging
    • often start with abdominal ultrasound or MRI/MRCP
    • MRI, MRCP, or MDCT are most helpful for visualizing intrahepatic lesion
    • EUS or ERCP are most helpful for visualizing extrahepatic lesions
Treatment
  • Surgery 
    • may be curative if no lymph node, vascular, or distant metastatic spread
    • few patients present at early stage 
  • Chemotherapy and radiation
    • may be used in adjuvant, neo-adjuvant, and/or palliative settings

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