In addition to PPGLs, other tumors, including renal cell carcinoma (RCC), GIST, pituitary adenoma, pulmonary chondroma (as part of Carney triad, which also includes PGL and GIST), and papillary thyroid cancer, as well as thyroid nodules have been reported in patients with SDHx mutations or SDHC epimutation; estimated penetrance of RCC in SDHx carriers is 2-3% (mostly SDHB), but 85% of GISTs diagnosed in childhood are related to SDHx mutations (4, 7, 48). This evidence concerns the gene SDHB and renal cell carcinoma.