Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
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DOI：
10.1001/jamanetworkopen.2021.24750
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发表时间：
2021-09-01
影响因子：
13.8
通讯作者：
Luo F
中科院分区：
文献类型：
--
作者：
Xu Z;Wang L;Dai S;Chen M;Li F;Sun J;Luo F
美国胃肠胰腺神经内分泌肿瘤（GEP-NETs）的流行病学趋势和预后因素是什么？在43751例GEP-NET患者的队列研究中，大多数病例发生在直肠和小肠。从1975年到2015年，GEP-NET的年龄调整发病率显著增加，尤其是直肠、局部和1级GEP-NET;年龄、性别、婚姻状况和肿瘤大小、级别、分期和部位与GEP-NET患者的总生存期显著相关。GEP-NET的发病率和患病率在40年中持续增加;本研究表明，基于预后因素的预测模型可以准确量化GEP-NET患者的死亡风险，表明该模型具有令人满意的临床实用性。虽然胃肠胰腺神经内分泌肿瘤（GEP-NETs）的发病率和患病率在过去几十年中一直被认为有所增加，但缺乏最新的流行病学和生存数据。使用最新数据对最大的GEP-NET患者队列进行流行病学和生存分析，并建立一种新的列线图来预测GEP-NET个体患者的生存概率。在这项队列研究中，从1975年1月1日至2015年12月31日诊断的43751例GEP-NET患者中确定了监测，流行病学和最终结果计划。相关数据用于流行病学和生存分析，以及建立和验证诺模图，以预测GEP-NET患者的生存概率。研究截止日期为2018年12月31日。统计分析时间为2020年2月1日至4月30日。GEP-NET患者的发病率、总生存率相关因素和列线图模型从1975年到2015年，共有43751名患者接受了GEP-NET的诊断（22 398例女性[51.2%]，31 976例白色患者[73.1%]，7097例黑人患者[16.2%]，3207例亚洲和太平洋岛民患者[7.3%]，270例美洲印第安人和阿拉斯加原住民患者[0.6%]，4546例患者的种族未知[10.4%];诊断时的平均[SD]年龄为58 [15]岁）。从1975年到2015年，GEP-NET的年龄调整发病率增加了6.4倍（年百分比变化[APC]，4.98; 95% CI，4.75-5.20; P < .001）。此外，在部位组中，直肠GEP-NET的发生率增加最显著（APC，6.43; 95%CI，5.65-7.23; P <0.001）。至于分期和分级，局限性GEP-NET（APC，6.53; 95%CI，6.08-6.97; P < .001）和G1 GEP-NET（APC，18.93; 95%CI，17.44-20.43; P < .001）的发病率增加最多。在研究期间，诊断为局限性疾病的平均年龄增加了9.0岁（95% CI，3.3-14.7岁; P = 0.002），区域和远处病例保持不变。在多变量分析中，年龄、性别、婚姻状况和肿瘤大小、分级、分期和部位与GEP-NET患者的总生存率显著相关（例如，远处与局限性疾病患者：风险比，10.32; 95%CI，8.56-12.43; G4与G1 GEP-NET：风险比，6.37; 95%CI，5.39-7.53）。此外，建立了一个包括年龄、大小、分级、分期和部位的诺模图来预测3年和5年生存概率，内部验证的一致性指数为0.893（95%CI，0.883-0.903），外部验证的一致性指数为0.880（95%CI，0.866-0.894）。受试者工作特征曲线显示诺模图比TNM分类具有更好的区分能力（3年总生存率的曲线下面积为0.908 vs 0.795; 5年总生存率的曲线下面积为0.893 vs 0.791）。在这项研究中，GEP-NET的发病率和患病率在40年内持续增加，特别是在直肠GEP-NET患者中。此外，本研究表明，具有5个预后参数的诺模图可以准确地量化GEP-NETs患者的死亡风险，表明其具有令人满意的临床实用性。这项队列研究使用来自监测、流行病学和最终结果项目的数据，对胃肠胰腺神经内分泌肿瘤患者进行流行病学和生存分析，并建立诺模图来预测这些患者的生存概率。
What are the epidemiologic trends of and prognostic factors for gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in patients in the US? In this cohort study of 43 751 patients with GEP-NETs, most cases occurred in the rectum and small intestine. The age-adjusted incidence rate of GEP-NETs increased significantly from 1975 to 2015, especially for rectal, localized, and grade 1 GEP-NETs; age, sex, marital status and tumor size, grade, stage, and site were significantly associated with overall survival for patients with GEP-NETs. The incidence and prevalence of GEP-NETs have continued to increase over 40 years; this study suggests that a predictive model based on prognostic factors may accurately quantify the risk of death among patients with GEP-NETs, indicating that the model has satisfactory clinical practicality. Although the incidence and prevalence of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) have been thought to have increased during the past decades, updated epidemiologic and survival data are lacking. To conduct an epidemiologic and survival analysis of the largest cohort of patients with GEP-NETs using the latest data and to establish a novel nomogram to predict the survival probability of individual patients with GEP-NETs. In this cohort study, 43 751 patients with GEP-NETs diagnosed from January 1, 1975, to December 31, 2015, were identified from the Surveillance, Epidemiology, and End Results Program. Associated data were used for epidemiologic and survival analysis, as well as the establishment and validation of a nomogram to predict the survival probability of individual patients with GEP-NETs. The study cutoff date was December 31, 2018. Statistical analysis was performed from February 1 to April 30, 2020. Incidence, factors associated with overall survival, and a nomogram model for patients with GEP-NETs. A total of 43 751 patients received a diagnosis of GEP-NETs from 1975 to 2015 (22 398 women [51.2%], 31 976 White patients [73.1%], 7097 Black patients [16.2%], 3207 Asian and Pacific Islander patients [7.3%], 270 American Indian and Alaska Native patients [0.6%], and 4546 patients of unknown race [10.4%]; mean [SD] age at diagnosis, 58 [15] years). The age-adjusted incidence rate of GEP-NETs increased 6.4-fold from 1975 to 2015 (annual percentage change [APC], 4.98; 95% CI, 4.75-5.20; P < .001). Furthermore, among site groups, the incidence of GEP-NETs in the rectum increased most significantly (APC, 6.43; 95% CI, 5.65-7.23; P < .001). As for stage and grade, the incidence increased the most in localized GEP-NETs (APC, 6.53; 95% CI, 6.08-6.97; P < .001) and G1 GEP-NETs (APC, 18.93; 95% CI, 17.44-20.43; P < .001). During the study period, the mean age at diagnosis for localized disease increased by 9.0 years (95% CI, 3.3-14.7 years; P = .002), which remained unchanged for regional and distant cases. On multivariable analyses, age, sex, marital status, and tumor size, grade, stage, and site were significantly associated with overall survival for patients with GEP-NETs (eg, patients with distant vs localized disease: hazard ratio, 10.32; 95% CI, 8.56-12.43; G4 vs G1 GEP-NET: hazard ratio, 6.37; 95% CI, 5.39-7.53). Furthermore, a nomogram comprising age, size, grade, stage, and site was established to predict the 3-year and 5-year survival probability, with the concordance indexes of 0.893 (95% CI, 0.883-0.903) for the internal validations and 0.880 (95% CI, 0.866-0.894) for the external validations. The receiver operating characteristic curve demonstrated that the nomogram exhibited better discrimination power than TNM classification (area under the curve for 3-year overall survival, 0.908 vs 0.795; for 5-year overall survival, 0.893 vs 0.791). In this study, the incidence and prevalence of GEP-NETs have continued to increase over 40 years, especially among patients with rectal GEP-NETs. In addition, this study suggests that a nomogram with 5 prognostic parameters may accurately quantify the risk of death among patients with GEP-NETs, indicating that it has satisfactory clinical practicality. This cohort study uses data from the Surveillance, Epidemiology, and End Results Program to conduct an epidemiologic and survival analysis of patients with gastroenteropancreatic neuroendocrine tumors and to establish a nomogram to predict the survival probability of these patients.
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