Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin. GIST spans a wide clinical spectrum that ranges from tumors with essentially no metastatic potential to malignant and life-threatening spread diseases. Gain-of-function mutations in KIT or PDGFRA receptor tyrosine kinases are the crucial drivers of most GISTs, responsible for tumor initiation and evolution throughout the entire course of the disease. The introduction of tyrosine kinase inhibitors targeting these receptors has substantially improved the outcomes in this formerly chemoresistant cancer. As of today, five agents hold regulatory approval for the treatment of GIST: imatinib, sunitinib, regorafenib, ripretinib, and avapritinib. This, in turn, represents a success for a rare neoplasm. During the past two decades, GIST has become a paradigmatic model in cancer for multidisciplinary work, given the disease-specific particularities regarding tumor biology and tumor evolution. Herein, we review currently available evidence for the management of GIST. This clinical practice guideline has been developed by a multidisciplinary expert panel (oncologist, pathologist, surgeon, molecular biologist, radiologist, and representative of patients' advocacy groups) from the Spanish Group for Sarcoma Research, and it is conceived to provide, from a critical perspective, the standard approach for diagnosis, treatment, and follow-up.

中文翻译：

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2023 年 GEIS 胃肠道间质瘤指南。

胃肠道间质瘤（GIST）是最常见的间质来源的恶性肿瘤。胃肠道间质瘤（GIST）涵盖广泛的临床范围，从基本上没有转移潜力的肿瘤到恶性和危及生命的传播疾病。KIT 或 PDGFRA 受体酪氨酸激酶的功能获得性突变是大多数 GIST 的关键驱动因素，负责整个疾病过程中肿瘤的发生和演变。针对这些受体的酪氨酸激酶抑制剂的引入大大改善了这种以前具有化学抗性的癌症的治疗结果。截至目前，有五种药物获得监管部门批准用于治疗 GIST：伊马替尼、舒尼替尼、瑞格非尼、瑞普替尼和阿瓦普替尼。这反过来又代表了一种罕见肿瘤的成功。在过去的二十年中，考虑到肿瘤生物学和肿瘤进化的疾病特异性，GIST 已成为多学科工作的癌症范例模型。在此，我们回顾了当前 GIST 治疗的可用证据。本临床实践指南由西班牙肉瘤研究小组的多学科专家小组（肿瘤学家、病理学家、外科医生、分子生物学家、放射科医生和患者倡导团体代表）制定，旨在提供从关键观点，诊断、治疗和随访的标准方法。