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胃肠道间质瘤:诊断与治疗

Gastrointestinal stromal tumors: diagnosis and treatment.

作者信息

Acín-Gándara Débora, Pereira-Pérez Fernando, Castaño-Pascual Angel, Durán-Poveda Manuel, Antequera-Pérez Alfonso, Miliani-Molina Carlos

机构信息

Cirugia General y de Aparato Digestivo, Hospital Universitario de Fuenlabrada, Madrid, Spain.

出版信息

Cir Cir. 2012 Jan-Feb;80(1):44-51.

Abstract

BACKGROUND

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the digestive tract. They originate from the interstitial cells of Cajal and are characterized by the overexpression of KIT protein (tyrosine kinase). Their prognosis has improved significantly with the discovery of imatinib mesylate for advanced GIST treatment.

METHODS

We carried out a retrospective, descriptive study of GISTs diagnosed in our center during the past 5 years. We excluded patients with incidental diagnoses in the context of other pathologies because GIST did not affect outcome or prognosis. The variables studied were clinical characteristics, location, size, imaging techniques, resectability, neoadjuvant imatinib, surgical technique, histology, immunohistochemistry, prognostic classification of Fletcher, morbidity, monitoring, and disease-free and overall survival.

RESULTS

Nineteen patients were diagnosed (14 males/5 females) with a mean age of 63 years (range: 30-84 years). Diagnosis was incidental in eight patients (42%). Tumor location of the remaining 11 patients (58%) was six tumors of the small intestine (55%), four gastric (36%) and one rectal (9%). Predominant gastrointestinal bleeding and anemia were diagnosed mainly by abdominal computed tomography (CT). At diagnosis, nine patients were considered resectable with radical intent (82%) and the other two patients (18%) received neoadjuvant treatment with a favorable response after 6 months. Three patients were treated with imatinib after surgery (33%). Median survival was 34 months (range: 5-58 months).

CONCLUSIONS

Diagnosis of GIST is often incidental. The predominant clinical symptom is usually gastrointestinal bleeding and anemia and the most widely used imaging test is CT. Treatment is surgical unless advanced GIST is diagnosed, which will be treated with imatinib mesylate neoadjuvant therapy. A multidisciplinary approach to this pathology is essential, a fact that affects prognosis and patient survival.

摘要

背景

胃肠道间质瘤(GIST)是消化道最常见的间叶性肿瘤。它们起源于 Cajal 间质细胞,其特征是 KIT 蛋白(酪氨酸激酶)过度表达。随着甲磺酸伊马替尼用于晚期 GIST 治疗的发现,其预后有了显著改善。

方法

我们对本中心在过去 5 年中诊断的 GIST 进行了一项回顾性描述性研究。我们排除了在其他病理情况下偶然诊断出的患者,因为 GIST 不影响结局或预后。研究的变量包括临床特征、位置、大小、成像技术、可切除性、新辅助伊马替尼治疗、手术技术、组织学、免疫组化、Fletcher 预后分类、发病率、监测以及无病生存期和总生存期。

结果

共诊断出 19 例患者(14 例男性/5 例女性),平均年龄 63 岁(范围:30 - 84 岁)。8 例患者(42%)为偶然诊断。其余 11 例患者(58%)的肿瘤位置为:小肠肿瘤 6 例(55%),胃肿瘤 4 例(36%),直肠肿瘤 1 例(9%)。主要的胃肠道出血和贫血主要通过腹部计算机断层扫描(CT)诊断。诊断时,9 例患者(82%)被认为可进行根治性切除,另外 2 例患者(18%)接受了新辅助治疗,6 个月后反应良好。3 例患者术后接受了伊马替尼治疗(33%)。中位生存期为 34 个月(范围:5 - 58 个月)。

结论

GIST 的诊断通常是偶然的。主要临床症状通常是胃肠道出血和贫血,最常用的成像检查是 CT。除非诊断为晚期 GIST,否则治疗以手术为主,晚期 GIST 将采用甲磺酸伊马替尼新辅助治疗。对这种疾病采用多学科方法至关重要,这一事实会影响预后和患者生存。

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