Maintenance therapy for pediatric sarcoma: full throttle ahead?

Purpose of review Maintenance therapy (MT), particularly antiangiogenic approaches such metronomic chemotherapy (MC), correspond to the continuous administration of low-dose anticancer agents in a context of minimal residual disease. While widely used in pediatric acute lymphoblastic leukemia for decades, MT has recently shown promise in solid tumors. Additionally, antivascular endothelial growth factor (VEGF) tyrosine kinase inhibitor (TKI) are increasingly explored in pediatric sarcomas. Recent findings This review summarize current evidence on MT efficacy in pediatric sarcomas, focusing on MC and TKIs. It examines their impact on the tumor microenvironment and cancer progression, as well as potential future applications, including standalone use or combination with targeted therapies, immunotherapies and/or drug repurposing. Summary MT has been demonstrated to improve outcomes in specific sarcomas, especially high-risk localized rhabdomyosarcoma, and has therefore become a standard of care. Its role in other sarcomas, such as Ewing sarcoma and osteosarcoma, is under investigation. However, critical challenges remain, including optimizing drug selection, treatment duration, and patient stratification to maximize benefits.