Cholangiocarcinoma: Diagnosis and Management

Clin Liver Dis. 2020 Aug;24(3):421-436. doi: 10.1016/j.cld.2020.04.005. Epub 2020 May 29.

Abstract

Cholangiocarcinoma is a highly lethal biliary epithelial tumor that is rare in the general population but has increased rates in patients with primary sclerosing cholangitis (PSC). It is heterogenous, and management varies by location. No effective prevention exists, and screening is likely only feasible in PSC. Patients often present in an advanced state with jaundice, weight loss, and cholestatic liver enzymes. Diagnosis requires imaging with magnetic resonance cholangiopancreatography, laboratory testing, and endoscopic retrograde cholangiopancreatography. Potentially curative options include resection and liver transplant with neoadjuvant or adjuvant chemoradiation. Chemotherapy, radiation, and locoregional therapy provide some survival benefit in unresectable disease.

Keywords: Chemotherapy; Cholangiocarcinoma; Diagnosis; ERCP; Management; Resection; Transplant.

Publication types

  • Review

MeSH terms

  • Alkaline Phosphatase / blood
  • Bile Duct Neoplasms / diagnosis*
  • Bile Duct Neoplasms / pathology
  • Bile Duct Neoplasms / therapy*
  • Bile Ducts, Extrahepatic
  • Bile Ducts, Intrahepatic
  • Bilirubin / blood
  • Chemoradiotherapy, Adjuvant
  • Cholangiocarcinoma / diagnosis*
  • Cholangiocarcinoma / pathology
  • Cholangiocarcinoma / therapy*
  • Cholangiopancreatography, Magnetic Resonance
  • Cholangitis, Sclerosing / complications
  • Humans
  • Liver Transplantation
  • Risk Factors
  • gamma-Glutamyltransferase / blood

Substances

  • gamma-Glutamyltransferase
  • Alkaline Phosphatase
  • Bilirubin