SDHB mutations are also associated with the development of RCC (14%), gastrointestinal stromal tumors [GIST; 2%; isolated or associated with PGL (Carney dyad or Carney-Stratakis syndrome) or PGL and chondroma (Carney triad)] and pituitary adenomas (rare) [85–90]. This evidence concerns the gene SDHB and gastrointestinal stromal tumor.