Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the digestive tract, and are mainly driven by activating mutations in KIT (also known as CD117) or platelet-derived growth factor A (PDGFRA), accounting for 0.1–3% of all gastrointestinal tumors. Here, PDGFRA is linked to gastrointestinal stromal tumor.