近来，免疫检查点B7-H3（CD276）作为B7家族中具有免疫调节特性的成员，已被鉴定为难治性血液肿瘤和实体恶性肿瘤的免疫治疗新靶点。尽管关于B7-H3在脑恶性肿瘤中的研究有限，但对于探索其在该领域的治疗潜力越来越感兴趣。B7-H3在调节肿瘤微环境中的免疫细胞、癌相关成纤维细胞和内皮细胞的功能中起到了至关重要的作用，对形成促肿瘤的环境起到了贡献。该微环境促进了肿瘤细胞的无限增殖、增强新陈代谢、增加癌症干细胞性质和对标准治疗的耐药性。阻断B7-H3并终止其免疫抑制功能预期能够改善抗肿瘤免疫应答，并进而改善肿瘤的进展。预临床或观察性研究以及靶向B7-H3的初期临床试验结果表明，在胶质母细胞瘤（GBM）、漿液性脑桥脑瘤（DIPG）、髓母细胞瘤、神经母细胞瘤、颅咽管瘤、非典型畸胎瘤/纪要肉瘤肿瘤和脑转移瘤中，具有有希望的抗肿瘤疗效和可接受的毒性。正在进行的临床试验目前在对抗难治性或复发性GBMs、DIPGs、神经母细胞瘤、髓母细胞瘤、脑室管瘤和脑转移瘤中，通过CAR-T细胞疗法和抗体药物联合治疗，无论是单独应用还是结合标准治疗或其他治疗方法，探究B7-H3的使用。这些试验为成人和儿童人群中这些具有挑战性的颅内恶性肿瘤提供了有效的治疗选择。©作者。

The immune checkpoint B7-H3 (CD276), a member of the B7 family with immunoregulatory properties, has been identified recently as a novel target for immunotherapy for refractory blood cancers and solid malignant tumors. While research on B7-H3 in brain malignancies is limited, there is growing interest in exploring its therapeutic potential in this context. B7-H3 plays a crucial role in regulating the functions of immune cells, cancer-associated fibroblasts, and endothelial cells within the tumor microenvironment, contributing to the creation of a pro-tumorigenic milieu. This microenvironment promotes uncontrolled cancer cell proliferation, enhanced metabolism, increased cancer stemness, and resistance to standard treatments. Blocking B7-H3 and terminating its immunosuppressive function is expected to improve anti-tumor immune responses and, in turn, ameliorate the progression of tumors. Results from preclinical or observative studies and early-phase trials targeting B7-H3 have revealed promising anti-tumor efficacy and acceptable toxicity in glioblastoma (GBM), diffuse intrinsic pontine glioma (DIPG), medulloblastoma, neuroblastoma, craniopharyngioma, atypical teratoid/rhabdoid tumor, and brain metastases. Ongoing clinical trials are now investigating the use of CAR-T cell therapy and antibody-drug conjugate therapy, either alone or in combination with standard treatments or other therapeutic approaches, targeting B7-H3 in refractory or recurrent GBMs, DIPGs, neuroblastomas, medulloblastomas, ependymomas, and metastatic brain tumors. These trials hold promise for providing effective treatment options for these challenging intracranial malignancies in both adult and pediatric populations.© The author(s).