An update on gastrointestinal stromal tumors (GISTs) with a focus on extragastrointestinal stromal tumors (EGISTs).

IF 4.2 3区 医学 Q2 GASTROENTEROLOGY & HEPATOLOGY
Gastroenterology Report Pub Date : 2025-07-23 eCollection Date: 2025-01-01 DOI:10.1093/gastro/goaf068
Fatima Usama, Rohullah Rasikh, Khawaja Hassam, Mansoor Rahman, Fnu Khalil Ur Rehman, Iman Waheed Khan, Daryl T-Y Lau
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引用次数: 0

Abstract

Gastrointestinal stromal tumors (GISTs) originate from mesenchymal cells and account for ∼1% of primary malignant tumors in the digestive system. They are diagnosed based on characteristic immunohistochemical staining pattern, including CD117 and DOG1, as well as genetic analysis for mutations in the KIT and platelet-derived growth factor receptor α genes. Extragastrointestinal stromal tumors (EGISTs) share very similar morphology with GISTs but arise outside the gastrointestinal tract. The most common locations for EGISTs are the omentum, mesentery, retroperitoneum, and pancreas, followed by the liver, vagina, and prostate. The mean age of presentation of these tumors is in the sixth decade of life and tumor dimensions at different locations typically range from 7 to 15.8 cm. Most of these tumors are unifocal and of the spindle cell type. GISTs generally have a better prognosis than EGISTs, with cumulative 5-year survival rates of 85% for GISTs and 38%-60.9% for EGISTs. Among EGISTs, omental tumors have higher overall survival than mesenteric or retroperitoneal tumors. Additionally, age of >60 years, male sex, larger tumor size, higher mitotic rate, and nuclear pleomorphism are associated with worse prognosis in EGISTs.

胃肠道间质瘤(gist)的最新进展,重点是胃肠外间质瘤(egist)。
胃肠道间质瘤(gist)起源于间充质细胞,约占消化系统原发性恶性肿瘤的1%。它们的诊断基于特异性免疫组织化学染色模式,包括CD117和DOG1,以及KIT和血小板衍生生长因子受体α基因突变的遗传分析。胃肠外间质瘤(egist)与胃肠道间质瘤具有非常相似的形态,但发生在胃肠道外。egist最常见的部位是网膜、肠系膜、腹膜后和胰腺,其次是肝脏、阴道和前列腺。这些肿瘤出现的平均年龄在60岁左右,不同部位的肿瘤尺寸通常在7到15.8厘米之间。这些肿瘤多数为单灶性梭形细胞型。gist一般预后优于egist,其累积5年生存率为85%,egist为38%-60.9%。在egist中,大网膜肿瘤的总生存率高于肠系膜或腹膜后肿瘤。此外,年龄50 ~ 60岁、男性、较大的肿瘤大小、较高的有丝分裂率和核多形性与egist患者预后较差相关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Gastroenterology Report
Gastroenterology Report Medicine-Gastroenterology
CiteScore
4.60
自引率
2.80%
发文量
63
审稿时长
8 weeks
期刊介绍: Gastroenterology Report is an international fully open access (OA) online only journal, covering all areas related to gastrointestinal sciences, including studies of the alimentary tract, liver, biliary, pancreas, enteral nutrition and related fields. The journal aims to publish high quality research articles on both basic and clinical gastroenterology, authoritative reviews that bring together new advances in the field, as well as commentaries and highlight pieces that provide expert analysis of topical issues.
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