PDF(Pubmed)
Abstract：
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.
摘要：
胃肠道间质瘤（GIST）是最常见的间充质起源的恶性肿瘤。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受体酪氨酸激酶中的功能增益突变是大多数GIST的关键驱动因素，在整个疾病过程中负责肿瘤的发生和发展。靶向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化学抗性癌症的结果。截至今天,五种药物获得GIST治疗的监管批准：伊马替尼，舒尼替尼,Regorafenib,里替尼,和阿瓦替尼.这个,反过来,代表了罕见肿瘤的成功。在过去的二十年里，GIST已成为癌症多学科工作的典范模型，考虑到肿瘤生物学和肿瘤进化方面的疾病特异性。在这里，我们回顾了目前可用的GIST管理证据.本临床实践指南是由多学科专家小组(肿瘤学家，病理学家,外科医生,分子生物学家,放射科医生,和代表来自西班牙肉瘤研究小组的患者\'倡导团体)，它的构思是为了提供，从批判的角度来看,诊断的标准方法，治疗,和后续行动。
