Finally, germline genetic testing is recommended for every patient with SDH-deficient GIST and should be expanded to (1) every patient with GIST, (2) patients with cancer history especially if paraganglioma or adrenal lesions are present on imaging, (3) cancer family history of renal cell cancers or pheochromocytoma, and (4) somatic mutations in SDH(x). This evidence concerns the gene SDHB and paraganglioma.