...treated with irinotecan (90 mg/m2/dose) and adavosertib...Two of the three patients with objective responses had tumors with predicted ATRX deficiency, while the third did not have available archival tumor. Of the nine neuroblastoma patient tumors with predicted ATRX deficiency, 4 (44%) had objective responses or prolonged stable disease....The combination was of sufficient activity to support further study in neuroblastoma, and possibly other pediatric tumor types with recurrent ATRX mutation.