西北地区191例消化系统神经内分泌肿瘤回顾性分析
Retrospective analysis of 191 digestive system neuroendocrine neoplasm patients from the Northwest of China

摘要：目的 探索西北地区消化系统神经内分泌肿瘤(neuroendocrine neoplasm, NEN)的临床病理特征及其与生存预后的关系，为预防和诊治提供参考。方法 回顾性分析西安交通大学第一附属医院2006年1月至2015年12月被确诊为消化系统NEN患者的临床资料。所有纳入的患者均接受过手术切除，且均来自于西北地区。绘制Kaplan-Meier生存曲线并进行log-rank检验，将有统计学意义的因素引入Cox回归模型，从而得出影响患者生存预后的独立因素。结果 患者发病年龄在12～84岁，平均(55.3±12.9)岁，男女比例为3∶2。最常见的肿瘤原发部位是胃(62例，32.5%)，其次是胰腺(46例，24.1%)，功能性NEN仅占8.9%(17/191)。常用的NEN免疫组化标志物CgA、Syn、NSE、CK、CD56的阳性率分别为79.6%、94.9%、77.6%、81.9%、79.3%。58.5%的患者被检出时为Ⅲ/Ⅳ期，其中56.1%有淋巴结转移，24.0%有远处转移。根据WHO分类系统，71.2%的患者被诊断为低分化的神经内分泌癌(neuroendocrine carcinoma, NEC)。单因素分析结果显示，NEN患者的生存时间与TNM分期(P=0.002)、淋巴结转移(P=0.017)和术后辅助治疗(P<0.001)相关。多因素分析结果显示，TNM分期(P=0.040)和辅助治疗(P=0.001)是影响NEN患者生存预后的独立因素。结论 早期发现和辅助治疗使西北地区消化系统NEN患者受益。
ABSTRACT: Objective To investigate the clinicopathological characteristics and their relationship with the prognosis of neuroendocrine neoplasm (NEN) of the digestive system so as to provide reference for prevention, diagnosis and treatment of this disease. Methods We retrospectively reviewed 191 patients diagnosed with NENs of the gastrointestinal tract (GI) or hepato-biliary-pancreatic system (HBP) between January 2006 and December 2015 in The First Affiliated Hospital of Xi’an Jiaotong University. All the included patients underwent surgical resection and came from the Northwest of China. Kaplan-Meier survival curves were plotted and log-rank tests were performed to obtain the significant factors, which were later drawn into the Cox proportional hazards regression model for independent impact factors on the patient survival. Results Patients ranged in age from 12 to 84 years old (mean age of 55.3±12.9 years), and the male-to-female ratio was 3∶2. The most common primary tumor site was the stomach (n=62, 32.5%), followed by the pancreas (n=46, 24.1%). Of all the 191 cases, functional symptom accounted only for 8.9% (17/191). Based on the pathological reports, we observed that positive rates of immunohistochemical diagnosis of CgA, Syn, NSE, CK and CD56 were 79.6%, 94.9%, 77.6%, 81.9% and 79.3%, respectively. Most of the patients (58.5%) were diagnosed with stage III/IV NETs with lymphatic metastasis (56.1%) or distant metastasis (24.0%) according to AJCC stage and 137 (71.2%) patients were verified as G3 neuroendocrine carcinoma (NEC) according to the WHO classification. Univariate analysis showed that survival time of patients with NENs was significantly associated with TNM stage (P=0.002), lymphatic metastasis (P=0.017) and postoperative adjuvant therapy (AT)(P<0.001). Multivariate analysis showed that clinical stage (HR 0.547, 95% CI 0.307-0.974; P=0.04) and AT (HR 2.686, 95% CI 1.539-4.691;