The availability of multiple approved second-line targeted therapies, including agents directed against FGFR2 [4, 5], IDH1-mutations [6], ERBB2-/HER2 amplification/overexpression [7], MSI-high/dMMR [8], and tumor-agnostic therapies for NTRK-fusions [9], further underscores that CCA is not a single disease entity but a collection of diseases with distinct molecular subtypes (Fig. 1). This evidence concerns the gene IDH1 and cholangiocarcinoma.