Genetics and treatment of gastrointestinal stromal tumors with immune checkpoint inhibitors: what do we know?

Gastrointestinal stromal tumors (GIST) are mesenchymal neoplasms, derived from the interstitial cells of Cajal and mainly characterized from driver mutation in tyrosine kinase receptor genes KIT and PDGFRA. During the last 20 years, the tyrosine kinase inhibitors (TKI) revolutionized the therapy of GIST and resulted in extreme improvement in the survival of patients with metastatic disease. Unfortunately, responder patients invariably develop resistance over time to all TKI, imatinib as well as sunitinib and regorafenib. Recently, new TKI have demonstrated interesting results in the prolongation of survival in clinical studies