Approximately 20% of paediatric and adolescent/young adult patients with renal tumours are diagnosed with non-Wilms tumour, a broad heterogeneous group of tumours that includes clear-cell sarcoma of the kidney, congenital mesoblastic nephroma, malignant rhabdoid tumour of the kidney, renal-cell carcinoma, renal medullary carcinoma and other rare histologies. The differential diagnosis of these tumours dates back many decades, when these pathologies were identified initially through clinicopathological observation of entities with outcomes that diverged from Wilms tumour, corroborated with immunohistochemistry and molecular cytogenetics and, subsequently, through next-generation sequencing. These advances enabled near-definitive recognition of different tumours and risk stratification of patients. In parallel, the generation of new renal-tumour models of some of these pathologies including cell lines, organoids, xenografts and genetically engineered mouse models improved our understanding of the development of these tumours and have facilitated the identification of new therapeutic targets. Despite these many achievements, paediatric and adolescent/young adult patients continue to die from such rare cancers at higher rates than patients with Wilms tumour. Thus, international coordinated efforts are needed to answer unresolved questions and improve outcomes.
Nature reviews. Urology. 2025 Jan 29 [Epub ahead of print]
Daniela Perotti, Maureen J O'Sullivan, Amy L Walz, Jonathan Davick, Reem Al-Saadi, Daniel J Benedetti, Jack Brzezinski, Sara Ciceri, Nicholas G Cost, Jeffrey S Dome, Jarno Drost, Nicholas Evageliou, Rhoikos Furtwängler, Norbert Graf, Mariana Maschietto, Elizabeth A Mullen, Andrew J Murphy, Michael V Ortiz, Justine N van der Beek, Arnauld Verschuur, Jenny Wegert, Richard Williams, Filippo Spreafico, James I Geller, Marry M van den Heuvel-Eibrink, Andrew L Hong
Predictive Medicine: Molecular Bases of Genetic Risk, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy., Histology Laboratory, Children's Health Ireland at Crumlin, Dublin, Ireland., Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA., University of Iowa Hospitals and Clinics Stead Family Children's Hospital, Carver College of Medicine, Iowa City, IA, USA., UCL Great Ormond Street Institute of Child Health, University College London, London, UK., Division of Pediatric Hematology/Oncology, Vanderbilt University Medical Center, Nashville, TN, USA., Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada., Department of Surgery, Division of Urology, University of Colorado School of Medicine and the Surgical Oncology Program at Children's Hospital Colorado, Denver, CO, USA., Division of Oncology, Center for Cancer and Blood Disorders, Children's National Hospital, Washington, DC, USA., Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands., Children's Hospital of Philadelphia, Philadelphia, PA, USA., Pediatric Hematology and Oncology, Children's Hospital, Inselspital Bern University, Bern, Switzerland., Department Paediatric Oncology & Hematology, Saarland University, Homburg, Germany., Research Center, Boldrini Children's Hospital, Campinas, SP, Brazil., Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA., St. Jude Children's Research Hospital Memphis, Memphis, TN, USA., Memorial Sloan Kettering Cancer Center, New York, NY, USA., Department of Pediatric Hematology and Oncology, Hôpital d'Enfants de la Timone, APHM, Marseille, France., Theodor-Boveri-Institute/Biocenter, Developmental Biochemistry, Wuerzburg University, Wuerzburg, Germany., Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK., Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy., Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA., University Medical Center Utrecht, Utrecht, the Netherlands., Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA. .