Neuroblastoma is one of the most aggressive cancer forms in children, with highly heterogenous clinical manifestations ranging from spontaneous regression to high metastatic capacity. High-risk neuroblastoma has the highest mortality rates of all pediatric cancers, highlighting the urgent need for effective novel therapeutic interventions. B7-H3 immune checkpoint protein is highly expressed in neuroblastoma, and it is involved in oncogenic signaling, tumor cell plasticity, and drug resistance. Immunotherapies based on immune checkpoint inhibition have improved patient survival in several human cancers, and recent reports provide preclinical evidence on the benefits of targeting B7-H3 in neuroblastoma, with emphasis on novel CAR T/NK-cell approaches. Here, we summarize the current status of neuroblastoma targeted therapies, with a focus on B7-H3 as a promising novel immunoregulatory therapeutic target for high-risk neuroblastoma.
Keywords: B7-H3; CAR therapy; CD276; Immune checkpoint protein; Immunotherapy; Neuroblastoma.
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