The purpose of this study was to propose an update of the French guidelines from the national committee ccAFU on upper tract urothelial carcinomas (UTUC).

A systematic Medline search for epidemiology, risk factors, diagnosis, prognosis, treatment options and follow-up of UTUC was performed between 2022 and 2024 to evaluate available references and their levels of evidence.

UTUC is a rare malignancy with specific risk factors, including exposure to aristolochic acid and Lynch syndrome. Its diagnosis is based on the use of computed tomography urography and ureterorenoscopy with biopsies. A prognostic classification has been proposed to discriminate low- and high-risk lesions mainly on the basis of tumour grade (low/high) and imaging aspects (non-invasive/invasive). Tumour size (<2/≥2cm), focality (uni-/multifocal), and hydronephrosis (absent/present) are more relative risk factors. The standard of care for low-risk patients is currently conservative treatment involving ureterorenoscopy laser ablation, whereas radical nephroureterectomy (RNU) is advised for those with high-risk disease. Adjuvant treatments after RNU include intravesical instillation of chemotherapy to decrease the risk of bladder recurrence and delivery of platinum-based chemotherapy in pT2-T4 and/or pN+ patients. First-line treatment for metastatic UTUC is based on the combination of enfortumab vedotin plus pembrolizumab. For unfit patients, platinum-based chemotherapy plus nivolumab followed by maintenance nivolumab or platinum-based chemotherapy followed by maintenance avelumab in those with at least stable disease can be proposed. The surveillance regimen and schedule depend on the disease stage.

These updated guidelines will contribute to improving the management of patients diagnosed with UTUC.