Peri-operative management considerations for localised upper tract urothelial carcinoma in the setting of Lynch syndrome.

Lynch syndrome (LS)-associated upper tract urothelial cancer (UTUC) is an overall rare malignancy comprising only 5%-10% of all urothelial carcinomas. Given its rarity, specific management guidelines for LS-associated UTUC do not exist. The optimal peri-operative management of localised LS-UTUC remains unknown, and with future approvals, immunotherapy may allow for organ preservation. This report describes a case of localised LS-UTUC with prior history of stage I colon cancer. An initial attempt to obtain an insurance approval for pembrolizumab given LS and context of deficient mismatch repair was unsuccessful. She subsequently received neoadjuvant gemcitabine and split-dose cisplatin, leading to a complete pathological response, ypT0N0.