Introduction: The Evolving Landscape of High-Risk Neuroblastoma
High-risk neuroblastoma (HR-NBL) continues to represent one of the most significant challenges in pediatric oncology, accounting for approximately 15% of all pediatric cancer deaths. Despite intensive multimodal therapy—including induction chemotherapy, surgical resection, high-dose chemotherapy with autologous stem cell rescue, and radiotherapy—long-term survival rates historically remained below 50%. The emergence of immunotherapy targeting the disialoganglioside GD2, which is nearly universally expressed on neuroblastoma cells, has fundamentally shifted the therapeutic paradigm. Among these agents, dinutuximab beta, a chimeric monoclonal antibody, has emerged as a critical component in both the consolidation and relapsed settings. Recent data from the ITCC-SIOPEN BEACON Immuno and HR-NBL1/SIOPEN trials provide definitive evidence for the clinical application of this agent, clarifying its synergy with chemotherapy and its optimal administration without adjuvant cytokines.
