Currently, germline testing is recommended for all SDH-deficient GIST including SDHA in some clinical practice guidelines (9, 25), but no clear protocol for genetic counseling and follow-up of SDHx variant carriers and relatives has been released, especially for those carrying a germline SDHA pathogenic variant not yet linked to well-defined hereditary syndrome. This evidence concerns the gene SDHB and gastrointestinal stromal tumor.