Twelve patients (3.7%) carried germline mutations in syndromic RCC-associated genes (VHL (7, 2.2%), FH (2, 0.6%), TSC2 (2, 0.6%), BAP1 (1, 0.3%), FLCN (1, 0.3%), TSC1 (1, 0.3%)). The gene discussed is TSC1; the disease is renal cell carcinoma.