Midostaurin (Rydapt)

Multikinase inhibitor (KIT, FLT3, PDGFR, PKC)

Response rate
60% ORR in AdvSM; 45% major response
Onset
Weeks–months
Route
Oral 100mg twice daily
Line
1st
IgM effect
N/A

Evidence summary

First FDA-approved KIT inhibitor for advanced SM (April 2017). In the landmark phase II trial (NEJM 2016), midostaurin achieved 60% ORR with 45% major responses in 89 evaluable AdvSM patients. Median OS 28.7 months. Active against both wild-type and D816V-mutated KIT. Less selective than avapritinib, with higher rates of GI toxicity.