Six autosomal dominant pheochromocytoma/paraganglioma syndromes have distinct clinical features and include neurofibromatosis type 1 (NF1), multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau syndrome (VHL), renal cell carcinoma with SDHB mutation, Carney triad, and Carney-Stratakis syndrome (CSS). This evidence concerns the gene SDHB and von Hippel-Lindau disease.