Several hereditary RCC syndromes have been described, including von Hippel-Lindau (VHL), Birt-Hogg-Dube ́ (BHD), hereditary papillary renal cell carcinoma (HPRC), hereditary leiomyomatosis RCC (HLRCC), succinate dehydrogenase kidney cancer (SDH-RCC), tuberous sclerosis (TS), and Cowden syndrome (CS). The gene discussed is SDHB; the disease is Cowden syndrome 1.