The other rare autosomal dominant disorders associated with chRCC include BAP1 tumor predisposition syndrome (BAP1 gene) [19], hereditary paraganglioma/pheochromocytoma (PGL/PCC) syndrome (SDH A/B/C/D genes) [20], Cowden syndrome (PTEN mutations) [21], and tuberous sclerosis (TSC1/2 mutations) [22]. The gene discussed is BAP1; the disease is Cowden disease.