Surg Pathol Clin. 2018 Jun;11(2):403-429. doi: 10.1016/j.path.2018.02.005.


This article focuses on cholangiocarcinoma, both intrahepatic and extrahepatic. The various classification schemes based on anatomic location, macroscopic growth pattern, microscopic features, and cell of origin are outlined. The clinicopathologic, immunohistochemical and molecular differences between intrahepatic cholangiocarcinoma and extrahepatic cholangiocarcinoma, as well as differences in the 2 subtypes of intrahepatic cholangiocarcinoma, are discussed. Finally, precursor lesions, prognosis, treatment, and promising new potential targeted therapies are reviewed.

Keywords: Bile duct; Cholangiocarcinoma; Extrahepatic; IDH1; IDH2; Intrahepatic.

Publication types

  • Review

MeSH terms

  • Bile Duct Neoplasms / diagnosis
  • Bile Duct Neoplasms / genetics
  • Bile Duct Neoplasms / pathology*
  • Bile Duct Neoplasms / therapy
  • Bile Ducts, Extrahepatic
  • Bile Ducts, Intrahepatic
  • Cholangiocarcinoma / diagnosis
  • Cholangiocarcinoma / genetics
  • Cholangiocarcinoma / pathology*
  • Cholangiocarcinoma / therapy
  • Diagnosis, Differential
  • Humans
  • Molecular Targeted Therapy / methods
  • Neoplasm Staging
  • Prognosis