Gastrointestinal stromal tumors (GISTs), the most common mesenchymal tumors of the gastrointestinal tract, result from constitutively activating mutations in two oncogenes from the receptor tyrosine kinase family, KIT or platelet-derived growth factor receptor-α (PDGFRA) [1, 2]. The gene discussed is PDGFRA; the disease is gastrointestinal stromal tumor.