[Excerpt from the 2023 European Association for the Study of the Liver practice guidelines: prevention, diagnosis, and treatment of intrahepatic cholangiocarcinoma].

Abstract

Intrahepatic cholangiocarcinoma (iCCA) occurs within the liver, between the bile duct and the secondary bile duct. It is the second most common primary liver cancer after hepatocellular carcinoma, and its incidence rate is increasing worldwide. The mortality rate is alarming because of its clinical asymptomatic features (often leading to delayed diagnosis), high invasiveness, and treatment resistance. Early diagnosis, molecular pathological biology, accurate staging, and personalized multidisciplinary treatment are current challenges for researchers and physicians. Furthermore, iCCA is difficult to treat due to its high heterogeneity at the clinical, genomic, epigenetic, and molecular levels. However, recent advances in molecular, surgical, and targeted therapies, along with the recognition that the etiology, risk factors, pathophysiology, molecular biology, and clinical management of iCCA differ completely from those of hilar cholangiocarcinoma (pCCA) and distal cholangiocarcinoma (dCCA), have prompted the International Liver Cancer Association and the European Association for the Study of the Liver to commission international specialized experts to draft evidence-based guidelines for physicians involved in the diagnosis, treatment, and prognosis. Herein, the key points of the guidelines are excerpted.