Urothelial carcinoma in a man with hereditary nonpolyposis colon cancer

Although most upper tract urothelial carcinomas are believed to be acquired, patients with hereditary nonpolyposis colon cancer (HNPCC) may have more than 20 times the risk of the normal population for these cancers. Certain mismatch repair mutations are now known to be associated with the disease. Screening and surveillance regimens are still evolving, but urinalysis, urine cytology, cystoscopy, and periodic upper tract imaging are the mainstays. HNPCC should be considered in any patient who develops an upper tract urothelial cancer or has a suggestive family history.