Intrahepatic cholangiocarcinoma as a unique subtype: key updates from current guidelines

heterogenous molecular signature (Fig. 1).Early diagnosis, molecular characterization, accurate staging and personalized interdisciplinary management remain key challenges in the treatment of iCCA.Given the heterogeneity of existing guidelines for the treatment of CCA, this short review provides a focused summary of current clinical recommendations (ESMO Guideline 2023; EASL-ILCA Guideline 2023; Deutsche S3-Leitlinie 2024) for the management of iCCA with particular emphasis on the EASL-guideline, the first to offer a dedicated framework specifically for iCCA. Epidemiology and classificationThe age standardised incidence rate of CCA remains low in Europe, the USA and Australasia ranging from 0.3 to 3.5 cases per 100,000 population (ESMO Guideline 2023).Despite this, the global mortality rate for CCA has increased over recent decades, as reported by the World Health Organization and Pan American Health Organization databases (ESMO Guideline 2023; Bertuccio 2019).While overall CCA incidence in Asia has remained stable or declined, the incidence of iCCA has shown a steady rise in most Western countries (ESMO Guideline 2023;Patel 2002;Saha 2016;Khan 2019).Age is a continuos and cumulative risk factor (Deutsche S3-Leitlinie).The highest CCA incidences are observed in south east Asia where liver fluke infections cause chronic cholangitis with local rates reaching up to 80 persons per 100,000 population (Banales 2016). Classification-macroscopicallyiCCA is classified into four subtypes: (a) "mass-forming " (MF), (b) periductal-infiltrating (PI), (c) MF + PI and (d) intraductal growing (EASL-ILCA Guideline).The latter is