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
Intrahepatic cholangiocarcinoma (iCCA) is a subgroup of cholangiocarcinoma that accounts for about 10%-20% of the total cases. Infection with hepatitis B virus (HBV) is one of the most important predisposing factors leading to the formation of iCCA. It has been recently estimated based on abundant epidemiological data that the association between HBV infection and iCCA is strong with an odds ratio of about 4.5. The HBV-associated mechanisms that lead to iCCA are under intense investigation. The diagnosis of iCCA in the context of chronic liver disease is challenging and often requires histological confirmation to distinguish from hepatocellular carcinoma. It is currently unclear whether antiviral treatment for HBV can decrease the incidence of iCCA. In terms of management, surgical resection remains the mainstay of treatment. There is a need for effective treatment modalities beyond resection in both first- and second-line treatment. In this review, we summarize the epidemiological evidence that links the two entities, discuss the pathogenesis of HBV-associated iCCA, and present the available data on the diagnosis and management of this cancer.
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.