Sonidegib increases the survival time in a mouse model of KIT D816V⁺ ASM and impairs MC proliferation when combined with midostaurin. (A) Survival analyses of NSG mice treated with sonidegib (n = 33; 80 mg/kg per day) or placebo (ctrl; n = 33) for 8 weeks, starting 7 weeks after transplantation with ROSA KIT D816V-gluc. (B) Mean survival time in NSG mice treated with sonidegib (80 mg/kg per day for 8 weeks). (C) Mean and median death of mice in ctrl vs sonidegib groups. The data are representative of 2 independent experiments. (D) Cell proliferation and death measured by flow cytometry (immunoprecipitation and annexin V) of HMC1.2 cell lines after 72 hours of treatment with sonidegib and/or PKC412. (E) Cell proliferation and death (table) of primary MCs of a patient with a KIT D816V⁺ ASM after 156 hours of treatment with sonidegib and/or PKC412. **P < .01.