ATRX altered PDX models LEY11 and LEY 16 were grafted into female CB-17/SCID mice and triaged to treatment with control or BAY1895344 (10 or 20 mg/kg daily)….Tumor growth inhibition was significantly greater in the BAY1895344 groups in both LEY 11 (n=12) and LEY 16 (n=13) (p=0.0003 and p = 0.006, respectively). Median overall survival was significantly longer in both LEY 11 (12.5 vs. 42 days, p = 0.001) and LEY 16 (32 vs. 60 days, p < 0.001)....BAY1895344 demonstrates promising in vivo activity against a PDX model of uLMS that harbors ATRX mutations, with no significant toxicity.