Patients with LS are 22 times more likely to develop UTUC than the general population [71] and have a lifetime risk of developing UTUC of up to 15–20%, with the highest risk among patients with MSH2 mutations (16% vs. 3% to 4% for MLH1 or MSH6) [2]. Here, MSH2 is linked to renal pelvis/ureter urothelial carcinoma.