胰腺神经内分泌肿瘤( PanNETs)是一种发病率低、异质性强的肿瘤.近年来,PanNETs偶然诊断逐年增多,临床上对这类疾病的治疗越来越受到关注. PanNETs的治疗方式较复杂尤其是晚期的PanNETs,新的治疗方式层出不穷.靶向治疗例如生长抑素类似物,mTOR抑制剂或血管生成抑制剂等,具有特异性强、副作用小等传统药物所不具有的优点,应用前景广阔.免疫治疗已经成为手术、放疗和化疗之后的又一种主要肿瘤治疗手段,PanNETs的免疫治疗主要涉及IFNα、免疫检查点抑制剂和溶瘤病毒等方面.本文介绍了关于神经内分泌肿瘤靶向治疗和新兴免疫疗法已完成或者正在进行的临床试验,并总结了靶向及免疫治疗的研究现状.“,”Pancreatic neuroendocrine tumors (PanNETs) are usually with low incidence and high heterogeneity. In re-cent years, incidental diagnosis of PanNETs greatly increased, and its treatment has received increasing attention. The treat-ment for PanNETs, especially advanced PanNETs, is relatively complicated, and new treatments are emerging. Targeted therapeutic drugs include somatostatin analogues, mTOR inhibitors or angiogenesis inhibitors, etc. Compared with traditional drugs, they have advantages such as strong specificity and mild side effects, and have broad application prospects. Immuno-therapy, after surgery, radiotherapy and chemotherapy, has become another major treatment of cancer. The immunotherapy for PanNETs mainly involves IFNα, immunological checkpoint inhibitors and oncolytic viruses, etc. This article describes the completed or ongoing clinical trials on targeted therapy and immunotherapy for neuroendocrine tumors, and has summa-rized the research status of targeted therapy and immunotherapy.