The SDH-GIST is resistant to all available tyrosine kinase inhibitors (TKIs) in most cases and may partly show transient stabilization or decrease in size under VEGFR inhibitor treatment because its progression is thought to be driven by the expression of insulin growth factor-1 receptor (IGF1R) and vascular endothelial growth factor receptor (VEGFR) induced by hypoxia-inducible factor-1α (HIF-1α) [4,40]. Here, SDHB is linked to gastrointestinal stromal tumor.