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胆囊神经内分泌肿瘤(GB-NET)较少见，早期临床表现不典型，也无特定的标志物，术前影像学难以与胆囊腺癌区分，确诊依靠病理及免疫组化检查，难以做到早期诊断。GB-NET恶性程度高，32.39%的患者在确诊时已经出现肝转移，56.10%的患者出现淋巴结转移，中位生存期仅为9～10个月。关于GB-NET的治疗目前没有专门的指南或共识可循，主要参照胆囊腺癌，根治性切除是首选治疗方案，新辅助化疗可能使一部分局部进展期患者肿瘤缩小达到可切除水平，术后辅助化疗有可能延长患者生存时间。本文回顾近年来GB-NET相关的临床和基础研究，结合国内外病例报道和SEER数据库中相关信息，对GB-NET的分类、临床病理特点、诊断、治疗进展及预后作一综述。“,”Neuroendocrine tumors of the gallbladder(GB-NET) are rare, and it lacks early clinical manifestations and has no specific tumor markers, it is difficult to distinguish GB-NET from gallbladder adenocarcinoma. The diagnosis of GB-NET is based on histopathology of the tumor and the assessment of proliferation fraction, which makes it difficult to achieve early diagnosis. GB-NET has a high degree of malignancy, 32.39% of patients have liver metastases at diagnosis, and 51.60% of patients have lymph node metastases, the median survival time is 9 to 10 months.There are currently no specific guidelines or consensus for the treatment of GB-NET. The treatment strategies are choosen mainly by the principles of gallbladder adenocarcinoma. We reviews the clinical and basic researches of GB-NET and case reports from China and across the world, as well as the data from SEER database, and we discuss the research progress on the classification, clinicopathological features, diagnosis, treatment advances and the prognosis.