PURPOSE: Upper-tract urothelial carcinoma (UTUC) is a relatively uncommon disease with limited available evidence on specific topics. The purpose of this article was to review the previous literature to summarize the current knowledge about UTUC epidemiology, diagnosis, preoperative evaluation and prognostic assessment. METHODS: Using MEDLINE, a non-systematic review was performed including articles between January 2000 and February 2016. English language original articles, reviews and editorials were selected based on their clinical relevance. RESULTS: UTUC accounts for 5-10 % of all urothelial cancers, with an increasing incidence. UTUC and bladder cancer share some common risk factors, even if they are two different entities regarding practical, biological and clinical characteristics. Aristolochic acid plays an important role in UTUC pathogenesis in certain regions. It is further estimated that approximately 10 % of UTUC are part of the hereditary non-polyposis colorectal cancer spectrum disease. UTUC diagnosis remains mainly based on imaging and endoscopy, but development of new technologies is rapidly changing the diagnosis algorithm. To help the decision-making process regarding surgical treatment, extent of lymphadenectomy and selection of neoadjuvant systemic therapies, predictive tools based on preoperative patient and tumor characteristics have been developed. CONCLUSIONS: Awareness regarding epidemiology, diagnosis, preoperative evaluation and prognostic assessment changes is essential to correctly diagnose and manage UTUC patients, thereby potentially improving their outcomes.

Department of Genitourinary Oncology Moffitt Cancer Center Tampa FL USA

Department of Pharmacological Sciences and Department of Medicine Stony Brook University Stony Brook NY USA

Department of Surgery McGill University Montreal QC Canada

Department of Urology Bern University Hospital Bern Switzerland

Department of Urology Caritas St Josef Medical Centre University of Regensburg Regensburg Germany

Department of Urology Clinica Alemana Santiago Chile

Department of Urology Faculty Hospital Motol 2nd Faculty of Medicine Charles University in Praha Prague Czech Republic

Department of Urology Fudan University Shanghai Cancer Center Shanghai China

Department of Urology Keio University School of Medicine Tokyo Japan

Department of Urology Medical University of Vienna Vienna Austria

Department of Urology University Medical Center Hamburg Eppendorf Hamburg Germany

Department of Urology University of Texas Southwestern Medical Center Dallas TX USA

Department of Urology Weill Cornell Medical College New York NY USA

Division of Urology Department of Surgical Sciences San Giovanni Battista Hospital University of Studies of Torino Turin Italy

Karl Landsteiner Institute of Urology and Andrology Vienna Austria

Scott Department of Urology Baylor College of Medicine Houston TX USA

Urology Department University of Sao Paulo Medical School and Institute of Cancer São Paulo Brazil

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