Dinutuximab beta effectively treats Ewing sarcoma when combined with chemotherapy.

Ewing sarcoma is a rare and aggressive cancer of the bone and soft tissues primarily affecting children and young adults. Prognosis for patients with metastatic or recurrent disease remains poor despite intensive multimodal therapy, highlighting the need of novel therapeutic approaches. The disialoganglioside GD2 is highly expressed on Ewing sarcoma cells, making this tumor eligible for anti-GD2 immunotherapy with dinutuximab beta. Through in vitro and in vivo approaches, this study demonstrated that dinutuximab beta effectively suppressed tumor growth by 60% (p = 0.0135) and improved survival rate by 68% (p = 0.0006) in a mouse model xenograft. The combination therapy with doxorubicin demonstrated superior efficacy compared to monotherapy, with enhanced tumor suppression (86%; p = 0.0009) and an extension of survival rate (146%; p = 0.000025). This study showed that dinutuximab beta, particularly in combination with standard chemotherapy, offers a promising approach to improve outcomes for high-risk Ewing sarcoma patients, providing a more effective alternative to current treatments.