The highest risk of urothelial cancer is observed in patients with pathogenic germline variants in MSH2 with cumulative risk estimates at age 70 of 5.8–6.9% for upper urinary tract (ureter and renal pelvis) cancer and 2.6–12.3% for bladder cancer, compared to 2.2–4.8% and 0–10.8% for those harboring MLH1 pathogenic variants and 0–2.9% and 0–1.7% for those harboring MSH6 pathogenic variants for upper urinary tract and bladder cancers, respectively (2, 4). Here, MSH2 is linked to urinary bladder cancer.