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胃肠道间质瘤:从偶然发现到急性胃肠道出血的广泛临床谱

[Gastrointestinal stromal tumors: a broad clinical spectrum from incidental -discovery to acute gastrointestinal bleeding].

作者信息

Siewert E, Tietze L, Maintz C, Geier A, Dietrich C G, Matern S, Gartung C

机构信息

Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Germany.

出版信息

Z Gastroenterol. 2004 Mar;42(3):233-42. doi: 10.1055/s-2004-812729.

Abstract

Three cases of gastrointestinal stromal tumors (GIST) are reported as typical examples of the broad clinical spectrum in which these rare tumors can be detected. The first case describes an 82-year-old patient with a hemorrhagic shock due to upper gastrointestinal bleeding from a GIST of the stomach. GIST most frequently present with either gastrointestinal bleeding, abdominal pain or a detectable mass on physical examination or by ultrasound imaging. Clinically asymptomatic tumor growth also occurs as demonstrated by the second case of a 44-year-old -woman with an incidental finding of GIST during surgery of the esophagus. The cases are used to discuss the consequences for therapy and prognosis resulting from the heterogeneity of this tumor entity; the relevant immunohistochemical markers used to distinguish between various tumor subtypes of gastrointestinal mesenchymal tumors (GIMT) are listed. Since gastrointestinal stromal tumors (GIST) represent the most common subgroup of GIMT, we focus on the clinicopathological prognostic factors of GIST. The third case of a 40-year-old patient with a malignant GIST recurrence after surgery and exhibiting secondary resistance after one year of successful therapy with the receptor tyrosine kinase inhibitor imatinib (Gleevec), antagonizing pathogenetically relevant constitutive c-KIT activation, illustrates the potential and limitations of the only effective drug treatment for advanced GIST.

摘要

报告了三例胃肠道间质瘤(GIST),作为可检测到这些罕见肿瘤的广泛临床谱的典型例子。第一例描述了一名82岁的患者,因胃GIST导致上消化道出血而发生失血性休克。GIST最常见的表现是胃肠道出血、腹痛,或在体格检查或超声成像时可检测到肿块。第二例是一名44岁女性,在食管手术中偶然发现GIST,这表明临床上也会出现无症状的肿瘤生长。这些病例用于讨论该肿瘤实体的异质性对治疗和预后的影响;列出了用于区分胃肠道间充质肿瘤(GIMT)各种肿瘤亚型的相关免疫组化标志物。由于胃肠道间质瘤(GIST)是GIMT最常见的亚组,我们重点关注GIST的临床病理预后因素。第三例是一名40岁患者,术后发生恶性GIST复发,在用受体酪氨酸激酶抑制剂伊马替尼(格列卫)成功治疗一年后出现继发性耐药,该药物可拮抗致病相关组成型c-KIT激活,这说明了晚期GIST唯一有效药物治疗的潜力和局限性。

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