The SDH-deficient GIST share a pathognomonic profile characterized by young age, female gender predilection, gastric localization, mixed epithelioid and spindle cell morphology, diffuse KIT and ANO1 (DOG1) IHC positivity, frequent lymph node metastatic involvement, and an indolent behaviour even often metastatic up-front [3–9]. This evidence concerns the gene SDHB and gastrointestinal stromal tumor.