Heinrich Michael C, Corless Christopher L
OHSU Cancer Institute, Oregon Health and Science University and VA Medical Center, Portland, Oregon 97239, USA.
J Surg Oncol. 2005 Jun 1;90(3):195-207; discussion 207. doi: 10.1002/jso.20230.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasm arising in the stomach. These tumors were previously classified as smooth muscle tumors, but in recent years it has become clear that they are clinically, pathologically, and molecularly distinct from other tumors and are much more common than previously appreciated. Historically, patients with primary localized or advanced GIST have been managed surgically, as there was no proven role of other treatment modalities such as radiation or chemotherapy. However, the field of GIST was revolutionized with the 1998 discovery that the vast majority of these tumors have oncogenic gain-of-function mutations of the KIT receptor tyrosine kinase. Follow-up studies have confirmed that KIT is both a useful diagnostic marker and an excellent therapeutic target. Imatinib, an inhibitor of KIT kinase activity, is now the standard front-line therapy for patients with advanced GIST. In this review, we discuss pathological and molecular features of gastric GISTs and review the historic and current roles of surgery in the treatment of patients with primary or metastatic GIST. The importance of a multi-disciplinary approach using both surgery and imatinib therapy is emphasized.
胃肠道间质瘤(GISTs)是起源于胃的最常见的间叶性肿瘤。这些肿瘤以前被归类为平滑肌肿瘤,但近年来已明确它们在临床、病理和分子水平上与其他肿瘤不同,且比以前认为的更为常见。从历史上看,原发性局限性或晚期GIST患者一直通过手术治疗,因为当时没有证据表明放疗或化疗等其他治疗方式有作用。然而,1998年发现绝大多数这些肿瘤具有KIT受体酪氨酸激酶的致癌性功能获得性突变,这彻底改变了GIST领域。后续研究证实KIT既是一种有用的诊断标志物,也是一个极佳的治疗靶点。伊马替尼,一种KIT激酶活性抑制剂,现在是晚期GIST患者的标准一线治疗药物。在这篇综述中,我们讨论胃GIST的病理和分子特征,并回顾手术在原发性或转移性GIST患者治疗中的历史和当前作用。强调了采用手术和伊马替尼治疗的多学科方法的重要性。