上消化道胃肠间质瘤：基于德国临床癌症研究组数据的流行病学、治疗和预后的基于人群分析。
Gastrointestinal stromal tumors of the upper GI tract: population-based analysis of epidemiology, treatment and outcome based on data from the German Clinical Cancer Registry Group.
机构信息
Department of Surgery, University Medical Center Schleswig-Holstein, Campus Lübeck, Ratzeburger Alle 160, 23564, Lübeck, Germany.
German Cancer Registry Group of the Society of German Tumor Centers - Network for Care, Quality and Research in Oncology (ADT), Berlin, Germany.
出版信息
J Cancer Res Clin Oncol. 2023 Aug;149(10):7461-7469. doi: 10.1007/s00432-023-04690-6. Epub 2023 Mar 23.
BACKGROUND
Gastrointestinal stromal tumors (GIST) are rare mesenchymal tumors. They are most frequently located in the stomach but are also found in the esophagus and the gastroesophageal junction (GEJ). Information regarding the prognostic factors associated with upper gastrointestinal GIST is still scarse.
METHODS
In this study, datasets provided by the German Clinical Cancer Registry Group, including a total of 93,069 patients with malignant tumors in the upper GI tract (C15, C16) between 2000 and 2016 were analyzed to investigate clinical outcomes of GIST in the entire upper GI tract.
RESULTS
We identified 1361 patients with GIST of the upper GI tract. Tumors were located in the esophagus in 37(2.7%) patients, at the GEJ in 70 (5.1%) patients, and in the stomach in 1254 (91.2%) patients. The incidence of GIST increased over time, reaching 5% of all UGI tumors in 2015. The median age was 69 years. The incidence of GIST was similar between males and females (53% vs 47%, respectively). However, the proportion of GIST in female patients increased continuously with advancing age, ranging from 34.7% (41-50 years) to 71.4% (91-100 years). Male patients were twice as likely to develop tumors in the esophagus and GEJ compared to females (3.4% vs. 1.9% and 6.7% vs. 3.4%, respectively). The median overall survival of upper gastrointestinal GIST was 129 months. The 1-year, 5-year, and 10-year OS was 93%, 79%, and 52% respectively. Nevertheless, tumors located in the esophagus and GEJ were associated with shorter OS compared to gastric GIST (130 vs. 111 months, p = 0.001). The incidence of documented distant metastasis increased with more proximal location of GIST (gastric vs. GEJ vs. esophagus: 13% vs. 16% vs. 27%) at presentation.
CONCLUSION
GIST of the esophagus and GEJ are rare soft tissue sarcomas with increasing incidence in Germany. They are characterized by worse survival outcomes and increased risk of metastasis compared to gastric GIST.
背景
胃肠道间质瘤（GIST）是一种罕见的间叶组织肿瘤。它们最常发生于胃，但也可发生于食管和胃食管交界处（GEJ）。目前关于与上胃肠道 GIST 相关的预后因素的信息仍然很少。
方法
本研究分析了德国临床癌症登记组提供的数据集，该数据集共包括 2000 年至 2016 年间 93069 例上胃肠道（C15、C16）恶性肿瘤患者，以探讨整个上胃肠道 GIST 的临床结局。
结果
我们确定了 1361 例上胃肠道 GIST 患者。肿瘤位于食管的 37 例（2.7%），位于 GEJ 的 70 例（5.1%），位于胃的 1254 例（91.2%）。GIST 的发病率随时间而增加，到 2015 年达到所有 UGI 肿瘤的 5%。中位年龄为 69 岁。男性和女性的 GIST 发病率相似（分别为 53%和 47%）。然而，女性患者中 GIST 的比例随着年龄的增长而不断增加，范围从 34.7%（41-50 岁）到 71.4%（91-100 岁）。与女性相比，男性发生食管和 GEJ 肿瘤的可能性是女性的两倍（分别为 3.4%和 6.7%比 1.9%）。上胃肠道 GIST 的中位总生存期为 129 个月。1 年、5 年和 10 年 OS 分别为 93%、79%和 52%。然而，与胃 GIST 相比，位于食管和 GEJ 的肿瘤与较短的 OS 相关（130 个月 vs. 111 个月，p=0.001）。初诊时，GIST 位置越靠近近端，记录的远处转移发生率越高（胃 vs. GEJ vs. 食管：13% vs. 16% vs. 27%）。
结论
德国食管和 GEJ 的 GIST 是罕见的软组织肉瘤，发病率呈上升趋势。与胃 GIST 相比，它们的生存结局较差，转移风险增加。
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