靶向免疫检查点的免疫治疗发展迅速，近年来在胃肠胰神经内分泌肿瘤（gastroenteropancreatic neuroendocrine neoplasm, GEP-NEN）中初步探索，但能否带来临床获益尚无定论。本文系统梳理免疫检查点抑制剂（immune checkpoint inhibitor，ICI）单药或双药联合治疗GEP-NEN的临床试验现状及疗效。结果表明，ICI在GEP-NEN中仍未取得突破性进展，治疗复发或转移性GEP-NEN有一定的抗肿瘤活性和安全性，但总体客观缓解率（objective response rate，ORR）较低。ORR与肿瘤分化程度呈负相关，分化差的胃肠胰神经内分泌癌可能更易获得临床缓解。双药与单药相比，疾病控制率更高，但不良反应更严重。鉴于错配修复基因缺陷和微卫星高度不稳定极为罕见，肿瘤突变负荷高（≥10 muts/Mb）的病人能从ICI免疫治疗中改善生存。未来期望进一步探索ICI与化疗、放疗、抗血管生成药物等在GEP-NEN的联合应用，有可能提高其抗肿瘤疗效，起到“1+1>2”的效果。临床应根据病理分化分级、免疫标志物、病情进展程度、病人身体状态等综合评估ICI免疫治疗的获益人群。

Immunotherapies targeting immune checkpoints have undergone rapid evolution, and have been preliminary explored in treatment of gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) in recent years. However, their potential to deliver tangible clinical benefits remains uncertain. In this article, we systematically reviewed the current status and efficacy of clinical trials, which evaluated immune checkpoint inhibitor (ICI) as monotherapy or in dual-ICI therapy for GEP-NEN. Despite lacking substantial breakthroughs in GEP-NEN treatment, ICI demonstrated some antitumor activity and safety in treating recurrent or metastatic GEP-NEN, albeit with a generally low objective response rate (ORR). The ORR of ICI in GEP-NEN treatment exhibited a negative correlation with tumor differentiation, suggesting that poorly diffe-rentiated gastroenteropancreatic neuroendocrine carcinoma (GEP-NEC) might achieve better clinical responses. Disease control rate of dual-ICI therapy was higher than that of monotherapy. However, dual-ICI also got more severe side effects. Given the rarity of mismatch repair gene defects and high microsatellite instability (dMMR/MSI-H) in GEP-NEN, patients with high tumor mutational burden (TMB-H≥10 muts/Mb) could get potentially benefit from ICI therapy. In the future, it is expected to further explore the synergistic combined application of ICI with chemotherapy, radiotherapy, and antiangiogenic drugs in GEP-NEN, which may enhance its antitumor efficacy. Clinically, the benefit groups of ICI immunotherapy should be evaluated comprehensively according to pathological grading, immune markers, disease progression, and patient's physical condition.