Our group reported the results from a phase-II clinical trial (NCT03951597) and showed combination therapy (Gemcitabine, Oxaliplatin, Lenvatinib, and anti-PD1 antibody, GOLP) as first-line therapy for patients with advanced iCCA had a promising ORR of 80% and median OS of 22.5 months10,11. This evidence concerns the gene PDCD1 and infantile convulsions and choreoathetosis.