Molecular target therapy for gastrointestinal stromal tumors

Gastrointestinal stromal tumors (GIST), the most common gastric mesenchymal tumor is unique due to the presence of a driver mutation called c-kit and the usage of imatinib as the targeted therapy. For resectable tumors, surgery is the preferred option and patients with high-risk GIST are considered for adjuvant imatinib for 3 years. The role of neoadjuvant imatinib is evolving. For the management of metastatic GIST, the FDA has approved imatinib, sunitinib and regorafenib as first, second and third line targeted therapy respectively. The increased prevalence of imatinib resistance has paved the way to the development of multiple other secondary and tertiary targeted agents. We present a brief review on the pathophysiology and resistance pathways and a comprehensive review of the various targeted agents which have been developed for the treatment of GIST.