Diagnosis and management of upper tract urothelial carcinoma

Urothelial carcinoma of the upper urinary tract (UTUC) is a rare disease, comprising only 5-10% of all urothelial malignancies. There is a paucity of high level evidence to guide treatment decisions due to the rarity of the disease. Through the creation of multi-institutional collaborations in recent years, our understanding of the natural history of UTUC and treatment algorithms has improved. However, our understanding of UTUC is mostly driven by extrapolation of findings and paradigms of urothelial carcinoma of the urinary bladder. With current imaging techniques and biopsy modalities, accurate diagnosis and staging remains difficult for this disease and prognostic models are limited in their ability to predict clinical outcomes. As such, over or under-treatment is common, highlighting the need for individualized treatment regimens which often require a multimodal approach. Endoscopic or radical resection represent the mainstays of treatment, while the role of intraluminal agents and systemic chemotherapy is yet to be clearly defined. Herein we review current concepts and management strategies as well as recent developments in UTUC.