Antitumor effect of FGFR inhibitors on a novel cholangiocarcinoma patient derived xenograft mouse model endogenously expressing an FGFR2-CCDC6 fusion protein.

•We established a new PDX mouse model bearing an FGFR2-CCDC6 fusion protein from a metastatic lung nodule of an iCCA patient.•FGFR inhibitors, ponatinib, dovitinib and BGJ398, inhibit FGFR signaling, and tumor growth in iCCAs harboring FGFR2 fusions.•Ponatinib was not additive or synergistic with standard gemcitabine and cisplatin chemotherapy on this PDX model.•There was a potential therapeutic advantage of BGJ398 over dovitinib and ponatinib in this model.