摘要目的 探讨中国神经内分泌肿瘤(NEN)、尤其消化系统NEN的发病部位和病理特征.方法 回顾性分析2012—2016年间就诊于卫生部中日友好医院所有NEN患者的临床病理资料,统计其病变部位分布,分析胃肠胰NEN肿瘤部位与病理分级的关系.结果 全组903例NEN患者,原发于消化系统者共699例(77.4%),原发于肺、胸腺或纵隔者87例(9.6%),其他部位60例(6.6%),原发灶不明者57例(6.3%).699例胃肠胰NEN中,原发于胃者207例(29.6%),胰腺201例(28.8%),直肠185例(26.5%),十二指肠43例(6.2%),空回肠18例(2.6%),阑尾15例(2.1%),胆囊11例(1.6%),食管10例(1.4%),结肠9例(1.3%);病理分级为神经内分泌瘤(NET)G1者336例(48.1%),NET G2者203例(29.0%),神经内分泌癌(NEC)G3者139例(19.9%).所有食管NEN(10/10)以及大多数胆囊(81.8%,9/11)和结肠(6/9)NEN为NEC(G3);所有阑尾NEN(15/15)以及大多数胃(71.0%,147/207)、胰腺(77.6%,156/201)、直肠(91.4%,169/185)、十二指肠(72.1%,31/43)、空回肠(88.9%,16/18)NEN为NET G1或G2.结论 消化系统NEN是最常见的NEN,其中胃、胰腺和直肠为最常见的胃肠胰NEN原发部位;不同的原发部位的胃肠胰NEN病理分级差异甚大,其中食管、结肠和胆囊NEN多为分化差的NEC.

abstractsObjective To explore the primary site and pathological feature of neuroendocrine neoplasm (NEN), especially the NEN of digestive system. Methods Clinicopathological data of NEN patients at China-Japan Friendship Hospital from January 2012 to December 2016 were retrospectively analyzed. Tumor primary sites were summarized. Association between tumor site and pathological grading in gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) was examined. Results There were a total of 903 cases of NEN. Sites of primary tumor included the digestive system in 699 cases(77.4%),the thorax (including lung, thymus and mediastinum) in 87 cases (9.6%), other sites in 60 cases (6.6%), unknown in 57 cases (6.3%). Among 699 GEP-NEN cases, the primary sites included the stomachin in 207 cases (29.6%), pancreas in 201 (28.8%), rectumin in 185 (26.5%), duodenum in 43 (6.2%), jejunum and ileum in 18 (2.6%), appendix in 15 (2.1%), gallbladder in 11 (1.6%), esophagus in 10 (1.4%), and the colon in 9 cases (1.3%). Pathologically, the tumor grading was neuroendocrine tumor (NET) G1 in 336 cases (48.1%), NET G2 in 203 cases (29.0%), neuroendocrine carcinoma (NEC) G3 in 139 cases (19.9%). All the esophagus NEN (10/10), most gallbladder NEN(9/11) and colon NEN(6/9) were poorly-differentiated NEC (G3), while all appendix NEN (15/15), most stomach NEN (147/207, 71.0%), pancreas NEN (156/201, 77.6%), rectum NEN (169/185, 91.4%), duodenum NEN (31/43, 72.1%), jejunum and ileum NEN (16/18, 88.9%) were well-differentiated NET G1 or G2. Conclusions The most common primary site of NEN is the digestive system. The stomach, pancreas and rectum are most common primary sitesof GEP-NEN. Difference in pathological grading is quite greatin different primary sites of GEP-NEN. Most NENs fromesophagus, colon and gallbladder are poorly-differentiated NEC.