SDH-deficient GISTs are the majority of pediatric GISTs in the stomach (110) and are part of two syndromes: the Carney triad (association of paranganglioma, pulmonary chondroma, and gastric GIST) and the Carney-Stratakis syndrome (association of GISTs and paragangliomas) (113, 117). This evidence concerns the gene SDHB and paraganglioma.