Within the SDH‐deficient GIST group, distinctive subgroups based on molecular and genetic aspects of the defect were identified.10 This group includes patients with the Carney‐Stratakis syndrome (gastric GIST and paragangliomas) carrying germline mutations in SDHB‐D, a subgroup of sporadic young adult cases with SDHA mutations as well as patients with epigenetic silencing of the SDHC gene mainly reported in syndromic Carney triad (gastric GIST, paragangliomas, pulmonary chondromas, and other tumors).10, 15, 16, 17. This evidence concerns the gene SDHB and paraganglioma.