Poveda Andrés, del Muro Xavier García, López-Guerrero Jose Antonio, Martínez Virginia, Romero Ignacio, Valverde Claudia, Cubedo Ricardo, Martín-Broto Javier
Instituto Valenciano de Oncología, Calle del Profesor Beltrán Bàguena, 8, 46009, Valencia, Spain,
Cancer Chemother Pharmacol. 2014 Nov;74(5):883-98. doi: 10.1007/s00280-014-2547-0. Epub 2014 Sep 6.
Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. Correct diagnosis with thorough use of pathologic and molecular tools of GIST mutations has been of the foremost importance. GIST are usually (95 %) KIT positive and harbor frequent KIT or platelet-derived growth factor receptor α-activating mutations. This deep molecular understanding has allowed the correct classification into risk groups with implications regarding prognosis, essential use in the development of targeted therapies and even response prediction to this drugs. Treatment has been evolving and an update to include lessons learned from recent trials in advanced disease as well as controversies in the adjuvant setting that are changing daily practice, is reviewed here. An effort from the Spanish Group for Sarcoma Research with investigators from the group has been undertaken to launch this third version of the GIST guidelines and provide a practical means for the different disciplines that treat this complex disease.
胃肠道间质瘤(GIST)是胃肠道最常见的间叶性软组织肉瘤。充分利用GIST突变的病理和分子工具进行正确诊断至关重要。GIST通常(95%)为KIT阳性,且常发生KIT或血小板衍生生长因子受体α激活突变。这种深入的分子认识使得能够正确分类为风险组,这对预后、靶向治疗开发中的必要应用甚至对这些药物的反应预测都有影响。治疗方法一直在不断发展,本文回顾了最新进展,包括从近期晚期疾病试验中吸取的经验教训以及辅助治疗中的争议,这些争议正在改变日常实践。西班牙肉瘤研究小组与该小组的研究人员共同努力推出了第三版GIST指南,为治疗这种复杂疾病的不同学科提供了一种实用方法。