[摘要] 表皮生长因子受体Ⅲ型突变体（epidermal growth factor receptor variant Ⅲ, EGFRvⅢ）是EGFR 最常见的突变体，在30%成胶质细胞瘤（glioblastoma multiforme, GBM）中表达。EGFRvⅢ源于外显子2～7 的缺失导致了受体的组成性激活，与肿瘤的发生、发展和不良预后密切相关。EGFRvⅢ独特的甘氨酸位点为靶向治疗提供了理想的分子靶点，针对EGFRvⅢ的免疫治疗策略在杀灭GBM细胞、抑制进展和侵袭等方面有着巨大的潜在价值，多肽/DC 疫苗、治疗性抗体、小分子抑制剂、过继细胞免疫治疗等在实验室和临床都取得了令人鼓舞的结果，本文对EGFRvⅢ的特点及GBM免疫治疗的研究进展予以评述。

[Abstract] EGFRvIII (epidermal growth factor receptor variant Ⅲ) is the most common mutant of epidermal growth factor receptor,which expresses in over 30% glioblastoma multiforme (GBM). EGFRvIII results from deletion of exon 2-7, leading to its constitutive activation, which is closely related to tumorigenesis, development and poor prognosis of GBM. The unique glycine site on EGFRvIII provides ideal molecular target for immunotherapy, which possess great potential value of killing GBM cell, inhibiting progress and invasion.Encouraging progress has been achieved in peptide/DC vaccine, therapeutic antibody, small molecular inhibitor and adoptive immunotherapy experimentally and clinically. The characteristics of EGFRvIII and the development in its oriented immunotherapy were summarized in this review.

国家自然科学基金资助项目（ No. 81372405, No.81772670）