自 2001 年实施 GIST 特异性组织学编码后，胃肠道间质瘤 (GIST) 的发病率有所增加，但缺乏趋势和生存率的更新数据。旨在检查主要器官部位 GIST 的流行病学演变。这种描述性的、基于人群的队列研究使用了来自国家癌症研究所监测、流行病学和最终结果 (SEER) 计划的全国代表性数据，包括 SEER-22 和 SEER-17 登记处。数据来自于2000年1月1日至2019年12月31日期间诊断出的20岁或以上患有GIST的评估患者。分析的最后更新日期为2023年10月29日。年龄标准化发病率和年度百分比变化的器官部位特定趋势(APC) 率按种族和族裔进行估计，并在可能的情况下按性别、年龄和主要指标进行估计。使用多变量 Cox 比例风险回归模型来检查按部位划分的总体生存率和 GIST 特异性生存率的种族和民族差异。SEER-22 和 SEER-17 数据集分别包含 23001 例和 12109 例 GIST 患者。 SEER-22 登记中的患者平均 (SD) 年龄为 64 (13) 岁，其中 51.3% 为男性。在种族和民族方面，9.7%的患者是亚裔或太平洋岛民，12.3%是西班牙裔，19.6%是非西班牙裔黑人，57.7%是非西班牙裔白人。 SEER-22 队列中除结肠外的所有器官部位的 GIST 总体发病率随着时间的推移大幅增加（APC：食道，7.3% [95% CI，4.4% 至 10.2%]；胃，5.1% [95% CI， 4.2% 至 6.1%]；小肠，2.7% [95% CI，1.8% 至 3.7%]；结肠，-0.2% [95% CI，-1.3% 至 0.9%]；直肠，1.9% [95%] CI，0.1% 至 3.8%]）。不同年龄组（<50 岁 vs ≥50 岁）、性别、种族和族裔以及胃和小肠 GIST 的主要指标也有类似的增长趋势。增加主要限于局部阶段疾病。 SEER-17 队列中患者的平均 (SD) 年龄为 64 (14) 岁，其中 51.9% 为男性。在种族和民族方面，13.3%的患者是亚裔或太平洋岛民，11.6%是西班牙裔，17.8%是非西班牙裔黑人，56.6%是非西班牙裔白人。与 GIST 相比，非西班牙裔黑人的食管（调整后风险比 [HR]，6.4 [95% CI，2.0 至 20.3]）和胃（调整后 HR，1.4 [95% CI，1.2 至 1.5]）GIST 总体死亡率较高非西班牙裔白人。亚洲或太平洋岛民的食管 GIST 总体死亡率也较高（调整后 HR，5.6 [95% CI，1.5 至 20.2]）。 GIST 特异性生存率的结果相似。在这项使用 SEER 数据的队列研究中，在过去 20 年中，几个人群中主要器官部位的 GIST 发病率有所增加。这些发现表明，有必要进行更多研究来确定危险因素，因为组织学重新分类以及内窥镜和影像学的更高可用性并不能完全解释这些不利的发生趋势。需要采取预防措施来减少少数种族和族裔人口之间巨大的生存差距。

The incidence of gastrointestinal stromal tumors (GISTs) increased after the implementation of GIST-specific histology coding in 2001, but updated data on trends and survival are lacking.To examine the evolving epidemiology of GISTs in major organ sites.This descriptive, population-based cohort study used nationally representative data from the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) Program, including the SEER-22 and SEER-17 registries. Data were from evaluated patients aged 20 years or older with GISTs diagnosed between January 1, 2000, and December 31, 2019. Analyses were last updated on October 29, 2023.Organ site-specific trends in age-standardized incidence rates and annual percent changes (APCs) in rates were estimated by race and ethnicity and, when possible, by sex, age, and primary indicator. Multivariable Cox proportional hazards regression models were used to examine racial and ethnic differences in overall and GIST-specific survival by site.The SEER-22 and SEER-17 datasets contained 23 001 and 12 109 case patients with GISTs, respectively. Patients in the SEER-22 registry had a mean (SD) age of 64 (13) years and 51.3% were men. With regard to race and ethnicity, 9.7% of patients were Asian or Pacific Islander, 12.3% were Hispanic, 19.6% were non-Hispanic Black, and 57.7% were non-Hispanic White. Overall incidence rates of GISTs in the SEER-22 cohort increased substantially over time for all organ sites but the colon (APCs: esophagus, 7.3% [95% CI, 4.4% to 10.2%]; gastric, 5.1% [95% CI, 4.2% to 6.1%]; small intestine, 2.7% [95% CI, 1.8% to 3.7%]; colon, -0.2% [95% CI, -1.3% to 0.9%]; and rectum, 1.9% [95% CI, 0.1% to 3.8%]). There were similar increasing trends by age groups (<50 vs ≥50 years), sex, race and ethnicity, and primary indicator for gastric and small intestine GISTs. Increases were mainly restricted to localized stage disease. Patients in the SEER-17 cohort had a mean (SD) age of 64 (14) years and 51.9% were men. With regard to race and ethnicity, 13.3% of patients were Asian or Pacific Islander, 11.6% were Hispanic, 17.8% were non-Hispanic Black, and 56.6% were non-Hispanic White. Non-Hispanic Black individuals had higher overall mortality for esophageal (adjusted hazard ratio [HR], 6.4 [95% CI, 2.0 to 20.3]) and gastric (adjusted HR, 1.4 [95% CI, 1.2 to 1.5]) GISTs compared with non-Hispanic White individuals. Asian or Pacific Islander individuals also had higher overall mortality for esophageal GISTs (adjusted HR, 5.6 [95% CI, 1.5 to 20.2]). Results were similar for GIST-specific survival.In this cohort study using SEER data, the incidence of GISTs in major organ sites increased in the last 2 decades among several population groups. These findings suggest that additional studies are warranted to identify risk factors, because histologic reclassification and higher availability of endoscopy and imaging do not fully explain these unfavorable incidence trends. Prevention efforts are needed to reduce the substantial survival disparities among racial and ethnic minoritized populations.