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目的:探究Vater壶腹部神经内分泌肿瘤(NETs)的流行病学特征、生物学行为和不同手术方式的远期效果。方法:检索SEER数据库中于1975—2017年被诊断为壶腹部NETs的病例,依据肿瘤恶性程度将其分为中低级别壶腹部NETs组与高级别壶腹部NETs组,分别比较其性别、年龄、肿瘤最大径、远处转移、总体生存期等临床病理特征的差异;在中低级别壶腹部NETs组中,比较局部切除和根治性切除对于患者的远期预后差异。采用n χ2检验比较两组患者年龄、性别、治疗方式、浸润深度、淋巴结转移、TNM分期、肝转移所占比例的差异;Kolmogorov-Smirnov正态分布检验法检验连续性变量;肿瘤最大径不符合正态分布,遂应用Wilcoxon秩和检验比较两组患者肿瘤最大径的差异;Log-rank法检验两组患者生存期的差异。n 结果:高级别壶腹部NETs组患者的中位发病年龄更高(67.61岁比61.73岁,n P=0.008)、确诊时肿瘤最大径更大(2.15 cm比1.50 cm,n P<0.001)、易发生肝转移(6.7%比24.5%,n P=0.001)、浸润深度更深(n P<0.001),且AJCC TNM分期更高(n P=0.006),总体中位生存期明显短于中低级别壶腹部NETs组(132个月比18个月,n P<0.001);病理上不同级别的NETs患者淋巴结转移率比较差异无统计学意义(n P=0.080);肿瘤最大径及浸润深度无法准确预测是否存在淋巴结转移;对于中低级别壶腹部NETs患者,接受局部切除者与扩大根治切除者预后总体生存期差异无统计学意义(n P=0.696)。n 结论:不同级别的壶腹部NETs生物学行为差异大,高级别壶腹部NETs恶性程度更高,易发生肝转移,预后不良;而中低级别壶腹部NETs恶性程度较低,预后较好,但淋巴结转移率与高级别壶腹部NETs无明显差异,且术前难以根据肿瘤级别、最大径或肿瘤浸润深度判断其是否存在淋巴结转移。尽管如此,对于中低级别壶腹部NETs,接受局部切除和扩大根治切除的患者预后并无明显差异。考虑到患者多为高龄,且胰十二指肠切除术较高的并发症发生率和病死率,对于术前未见明确淋巴结转移及远处转移的患者,推荐行局部切除术。“,”Objective:To explore the epidemiological characteristics, biological behaviors, and long-term survival of different surgical methods for neuroendocrine tumors (NETs) of ampulla of Vater.Methods:The SEER database was queried from 1975 to 2017 for patients diagnosed with ampullary NETs. Patients were divided into two groups according to the malignant grades of the tumor: the low-intermediate-grade NETs group and the high-grade NETs group. Their gender, age, tumor size, distant metastasis, overall survival time, and other clinicopathological differences were compared. In the low-intermediate-grade ampullary NETs group, the long-term prognosis of patients with local resection and radical resection were compared. The Chi-square test was used to compare the differences in the proportion of age, gender, method of treatment, depth of invasion, lymph node metastasis, TNM staging, and liver metastasis between the two groups; Kolmogorov-Smirnov test of normality was used to test continuous variables; tumor size was not normally distributed, then the Wilcoxon rank sum test was used to compare the difference in tumor size between the two groups; the Log-rank method was used to test the difference in survival time between the two groups.Results:For high-grade ampullary NETs, the median age of patients was higher (67.61 years n vs 61.73 years, n P=0.008), the median tumor size at diagnosis was larger (2.15 cm n vs 1.50 cm, n P<0.001), and higher liver metastases rates (6.7%n vs 24.5%, n P=0.001), the depth of infiltration was deeper (n P<0.001), and the AJCC TNM stage was higher (n P=0.006). The overall median survival of high-grade ampullary NETs was significantly shorter than that of low-intermediate-grade ampullary NETs (132 months n vs 18 months, n P<0.001). Pathologically, patients with different grades of NETs had no significant difference in the rate of lymph node metastasis (n P=0.080). Tumor size and the depth of infiltration could not accurately predict lymph node metastasis. For patients with low-intermediate-grade ampullary NETs, there was no significant difference in overall survival between those who received local resection and those who had radical surgery (n P=0.696).n Conclusions:The biological behaviors of ampullary NETs of different grades vary greatly, and high-grade ampullary NETs are more malignant, prone to liver metastasis, and have a poor prognosis. The low-grade or intermediate-grade NETs are associated with better prognosis, but the difference of lymph node metastasis rate is not significant, and it is difficult to judge whether there is lymph node metastasis based on the tumor grade, tumor infiltration or tumor size before surgery. Nevertheless, there is no significant difference in the prognosis of patients undergoing partial resection and radical resection in the low-intermediate-grade setting. Considering that the patients are mostly elderly, and the morbidity and mortality of pancreaticoduodenectomy are relatively high, local resection is recommended for patients with no evident lymph node metastasis and distant metastasis.