Combined Hepatocholangiocarcinoma: Unresolved Points

Combined hepatocholangiocarcinoma (cHCC-CCA) is an uncommon primary liver lesion in which aspects of hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) are intimately and unequivocally expressed. Its origin is still a topic of debate, given its heterogeneity, molecular profile, gene mutations, and also the complexities of the microenvironment in which it develops and the pathways that are triggered in the liver in relation to the phenomena of liver damage. Diagnosis and treatment are still a challenge, given the rarity of the disease and the complex heterogeneity of the tumor itself. Resective surgery remains the treatment of choice, but there is no strong recommendation regarding lymphadenectomy, with liver transplant still being controversial. There is still a lack of standards of care with regard to systemic therapy, but there are some promising findings on the efficacy of immune checkpoint inhibitors.