ANBL1821, A Phase 2 Randomized Study of Irinotecan/Temozolomide/Dinutuximab with or without Eflornithine (DFMO) (IND# 141913) in Children with Relapsed, Refractory or Progressive Neuroblastoma

This will be a prospective, randomized phase 2 trial to determine whether the addition of DFMo to the dinutuximab/irinotecan/temozolomide backbone improves the response rate compared to the backbone alone. Patients will be randomized 1:1 to Regimen a (irinotecan/ temozolomide/dinutuxumab) or Regimen B (DFMo + irinotecan/temozolomide/ dinutuximab), stratified by disease category (measurable vs. evaluable), prior anti-GD2 therapy (prior exposure to anti-GD2 antibody vs. no prior exposure to anti-GD2 antibody), prior DFMo therapy (prior exposure to DFMo vs. no prior exposure to DFMo), and MYCn status (amplified vs. non-amplified vs. unknown).