Published online Jul 21, 2021. doi: 10.3748/wjg.v27.i27.4252
Peer-review started: January 28, 2021
First decision: March 29, 2021
Revised: April 12, 2021
Accepted: June 18, 2021
Article in press: June 18, 2021
Published online: July 21, 2021
Core Tip: Cholangiocarcinoma (CCA) is the second most common primary liver cancer. Intrahepatic CCA (iCCA), a subgroup of CCA, has a pronounced association with hepatitis B virus (HBV) infection. Recent data have strengthened this association both in terms of epidemiology and pathogenesis. Moreover, the potential for the development of the combined hepatocellular-cholangiocarcinoma form poses additional difficulties in the successful management of iCCA. Herein, we discuss the available epidemiological data, present the current knowledge on the pathogenesis of HBV-associated iCCA, and review all of the available information on the diagnosis and management of this aggressive cancer.