Similar to bladder cancer, the cumulative lifetime risk, at 80 years of age, of developing hereditary UTUC is mutation-dependent, and strongly in favour of EPCAM/MSH2 germline variants (2.2–28%) compared to MSH6 and MLH-1 (0.2–5%) [29,35]. This evidence concerns the gene MSH2 and renal pelvis/ureter urothelial carcinoma.