摘要目的:探讨胃肠胰高增殖活性神经内分泌肿瘤（NET G3）的临床病理学特征，并对其预后进行分析。方法:收集复旦大学附属肿瘤医院2013年1月1日至2018年12月31日诊断的胃肠胰NET G3病例60例，总结分析临床病理特征和免疫组织化学结果，并进行生存相关的数据分析。同时收集2018年该院诊断的胃肠胰神经内分泌肿瘤（NEN）157例，比较各级NEN的发病比例。结果:60例胃肠胰NET G3中，男性32例，女性28例，年龄13~80岁，平均年龄54岁，其中胰腺原发最多见（48%，29/60），手术清扫病例中淋巴结转移比例达9/16，远处转移达41%（18/44），其中肝脏为最常见的转移部位（36%，16/44）。2018年该院诊断的胃肠胰NEN中，NET G3所占比率最低（7%，11/157）。显微镜下NET G3具有典型的分化好/中等的神经内分泌瘤病理形态特征，核分裂象计数病例之间差异显著。免疫组织化学染色显示大部分肿瘤细胞表达神经内分泌标志物，生长抑素受体2在60%病例中表达（12/20），Ki-67阳性指数平均值在30%~40%，不同病例之间Ki-67阳性指数差异显著（18%~80%）。胃肠胰NET G3总体生存时间为43个月，无病生存时间为12个月。结论:胃肠胰NET G3是最少见的一类NEN，具有独特的临床病理特征，预后较好。胃肠胰NEN的病理分类及分级能帮助临床选择合适的治疗并准确评估预后。

abstractsObjective:To investigate the clinicopathological features and outcome of gastroenteropancreatic high-grade neuroendocrine tumors.Methods:A total of 60 gastroenteropancreatic high-grade neuroendocrine tumors were collected from January 1st, 2013 to December 31th, 2018 at Fudan University Shanghai Cancer Center, with available pathology databases and clinic follow-up information. At the same time, 157 cases of gastrointestinal pancreatic neuroendocrine neoplasm (NEN) diagnosed at the hospital in 2018 were collected and the incidence of NEN at all grades was compared.Results:There were 32 males and 28 females, aged 13-80 years (mean 54 years). Pancreas primary was the most common (48%, 29/60). Nodal metastatic rate was 9/16 and distant metastatic rate was 41%(18/44). Liver was the most common site of metastasis. Among all the gastroenteropancreatic neuroendocrine neoplasms diagnosed in the hospital in 2018, the incidence of high-grade neuroendocrine tumors was the lowest (7%, 11/157). High-grade neuroendocrine tumors had typical pathologic features of well-differentiated/moderate neuroendocrine tumors, but with significant differences in mitotic rates. By immunohistochemical staining, most of the tumors expressed neuroendocrine markers and somatostatin receptor 2 was positive in 60% (12/20) of the cases. The average Ki-67 index was 30%-40%, and there was significant difference between cases (18%-80%). The overall survival of high-grade neuroendocrine tumors was 43 months, and the disease-free survival was 12 months.Conclusions:High-grade neuroendocrine tumor is a rare group of neuroendocrine tumors, with unique clinicopathological features and good prognosis. Pathological classification and grading of gastroenteropancreatic neuroendocrine neoplasms can help clinicians to select appropriate treatment and accurately evaluate prognosis.