Abstract
Imatinib, sunitinib, and regorafenib have remained the standard therapy for metastatic GIST. Avapritinib (a novel TK inhibitor) has been newly approved as the first-line treatment of metastatic GIST with a PDGFR D842 mutation, but has not been shown to be superior compared with regorafenib in the entire GIST population.
Other interesting tyrosine kinase inhibitors include cabozantinib, pazopanib, and ripretinib. Data on the efficacy of BRAF inhibitors and immunotherapy with anti-PD-1 drugs have been limited so far.
Repeated administration of imatinib after failure of previous treatment with imatinib, sunitinib, and regorafenib appears to have a minimum effect only. The recommended standard of 3-year adjuvant therapy with imatinib in patients after high-risk GIST resection has remained unchanged by the results of the PERSIST clinical trial with a 5-year administration of adjuvant imatinib.