儿童胃肠间质瘤治疗进展

胃肠道间质瘤(Gastrointestinal stromal tumors, GIST)是最常见的间叶源性肿瘤。最新统计结果显示,其全球发病率约5~19/106。成人患者临床表现常无特异性,患者多有腹痛、血便、腹胀等症状。GIST主要发生在中老年群体,在儿科极为罕见,因此儿童患者经常被漏诊或误诊。随着近年来基因检测的广泛应用,以及有关儿童GIST研究的深入,发现它代表着一个不同于成人GIST的临床疾病。成人GIST当前主要治疗方式以手术为主,同时根据患者病情应用靶向药物辅助治疗。然而对于儿科患者,一些用于成人肿瘤的治疗方案难以达到理想的治疗效果,甚至会造成不良后果。儿童胃肠道间质瘤的独特生物学特性,决定了它需要独特的治疗方案,以达到最佳的临床疗效。本综述旨在研讨最新文献,提供不同的儿童胃肠道间质瘤的治疗方式。

Progress in the Treatment of Gastrointestinal Stromal Tumors in Children

Gastrointestinal stromal tumors(GIST) is the most common tumor of mesenchymal origin.The latest statistical results showed that the incidence of the disease ranged from 5 to 19/106. Adult patient clinical expression often does not have specificity, the patient has the symptom such as abdominal pain, hematic defecate, abdominal distension. GIST mainly occurs in the middle-aged and elderly population, which is extremely rare in pediatrics, so pediatric patients are often missed or misdiagnosed. With the wide application of genetic testing in recent years and the in-depth study of GIST in children, it is found that it represents a clinical disease different from adult GIST. Currently, the main treatment modality for adult GIST is surgery, and targeted drug adjuvant therapy should be applied according to the patient’s condition. However, for pediatric patients, some adult tumor treatment programs are difficult to achieve the desired therapeutic effect, or even cause adverse consequences. The unique biological characteristics of gastrointestinal stromal tumors in children require a unique treatment regimen to achieve optimal clinical outcomes. The purpose of this review is to review the latest literature and to provide different approaches to the treatment of gastrointestinal stromal tumors in children.