Firstly, ivosidenib (AG-120) was tested in a phase I clinical trial enrolling 73 IDH-mutated CCA patients, 5% of which achieved partial response; median PFS and OS were 3.8 months (95% CI, 3.6–7.3) and 13.8 months (95% CI, 11.1–29.3), respectively [68]. This evidence concerns the gene IDH1 and cholangiocarcinoma.