Cao Hui, Wang Ming
Department of General Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China.
Zhonghua Wei Chang Wai Ke Za Zhi. 2014 Apr;17(4):312-6.
Gastrointestinal stromal tumors(GIST) are the most common mesenchymal tumors of the gastrointestinal tract, and are mostly characterized by c-kit or PDGFRA gain-of-function mutation. About 10%-15% of GIST do not harbor any mutations in the c-kit and PDGFRA genes and are defined as wild-type GIST. There are significant differences in molecular mechanism and clinical characteristics between wild-type GIST and mutant GIST. Wild-type GIST should be considered as a family of diseases due to their extreme heterogeneity. Clinician should pay close attention to the diagnosis and treatment of wild-type GIST.
胃肠道间质瘤(GIST)是胃肠道最常见的间叶组织肿瘤,大多具有c-kit或血小板衍生生长因子受体A(PDGFRA)功能获得性突变的特征。约10%-15%的GIST在c-kit和PDGFRA基因中未发生任何突变,被定义为野生型GIST。野生型GIST与突变型GIST在分子机制和临床特征上存在显著差异。由于野生型GIST具有极高的异质性,应将其视为一类疾病。临床医生应密切关注野生型GIST的诊断和治疗。