Intrahepatic cholangiocarcinoma: Epidemiology, risk factors, diagnosis and surgical management

Cancer Lett. 2016 Sep 1;379(2):198-205. doi: 10.1016/j.canlet.2015.09.008. Epub 2015 Sep 25.


Intrahepatic cholangiocarcinoma (ICC), the least common form of cholangiocarcinomas, is a rare hepatobiliary malignancy that arises from the epithelial cells of the intrahepatic bile ducts. The incidence of ICC has been rising in the global scale over the last twenty years, which may reflect both a true increase and the trend of earlier detection of the disease. Other than some well recognized causative risk factors, the association between viral and metabolic factors and ICC pathogenesis has been increasingly identified recently. Surgical resection is currently the only feasible modality with a curative ability, but the resectability and curability remain low. The high invasiveness of ICC predisposes the tumors to multifocality, node metastasis and vascular invasions, leading to poor long-term survival after resection. The role of liver transplantation is controversial, while locoregional treatments and systematic therapies may provide survival benefits, especially in patients with unresectable and advanced tumors. The present review discussed the epidemiology, risk factors, surgical and multimodal management of ICCs, which mainly focused on the outcomes and factors associated with surgical treatment.

Keywords: Diagnosis; Epidemiology; Intrahepatic cholangiocarcinoma; Risk factor; Surgery.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ablation Techniques
  • Bile Duct Neoplasms / diagnosis
  • Bile Duct Neoplasms / epidemiology
  • Bile Duct Neoplasms / surgery
  • Biliary Tract Surgical Procedures*
  • Cholangiocarcinoma* / diagnosis
  • Cholangiocarcinoma* / epidemiology
  • Cholangiocarcinoma* / surgery
  • Embolization, Therapeutic
  • Humans
  • Liver Transplantation
  • Neoplasm Staging
  • Predictive Value of Tests
  • Risk Factors
  • Treatment Outcome