Purpose of review: The aim of this study was to provide an update on the most recent developments regarding systemic treatments in the various molecular subtypes of gastrointestinal stromal tumour (GIST).
Recent findings: Several novel direct inhibitors of KIT and PDGFRA have entered the advanced clinical development in later treatment lines based on promising early clinical trial experience. Both avapritinib and ripretinib are more potent and more specific against various KIT and PDGFRA mutations. For patients with PDGFRA D842V mutations, the next generation of drugs may become the first active treatment options.Comprehensive molecular testing of KIT/PDGFRA-wildtype GIST may unmask clinically relevant targets, including NTRK fusions.
Summary: The treatment landscape in GIST is expected to undergo a profound transformation with more potent drugs currently in late-stage clinical development.