Abstract：Gastrointestinal stromal tumors are the most common type of sarcoma arising in the digestive tract, being commonly found in the stomach. Despite clinicopathological differences, most of the GISTs share oncogenic c-kit or platelet-derived growth factor-α(PDGFRA) mutations.Imatinib, c-kit and PDGFRA inhibitor, being successfully used in the treatment of GISTs and the response to the targeted treatment depend on the c-kit or PDGFRA mutation status. c-kit exon 11 mutants respond well to imatinib，while exon 9 mutants are less sensitive.GISTs withc-kit or PDGFRA secondary mutations will be resistant to imatinib. In this review, the correlation between c-kit and PDGFRA genes and clinical manifestation,molecular targeted therapy of GIST and the secondary c-kit and PDGFRA mutations will be elucidated.
