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Updated: Jul 28 2019


  • 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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