Is it a new era for intrahepatic cholangiocarcinoma?

Intrahepatic cholangiocarcinoma (iCCA) is increasingly considered as a separate entity from other biliary tract cancers (BTCs), due to differences in aetiology, risk factors, pathobiology, anatomical and molecular biology characteristics. Surgery is the only curative option for the ∼ 30 % who are diagnosed with a resectable disease, while liver-directed therapies (LDTs – i.e. hepatic-arterial-embolization, transarterial chemo-embolization, radio-embolization, and radiation therapy) and transplant are being explored and suggested by international guidelines as potential options of treatment for selected patients with unresectable liver-only disease. Standard of care first line treatment for advanced disease is chemoimmunotherapy, while there is growing evidence regarding the use of targeted treatment in second and further line, since up to 40 % of iCCA harbor a targetable molecular alteration. In the present review, the state of the art and future perspectives concerning the therapeutic management of iCCA at all stages is discussed.