Journal article
Hallmark discoveries in the biology of non-Wilms tumour childhood kidney cancers
Nature reviews. Urology, Vol.22(8), pp.526-543
08/2025
DOI: 10.1038/s41585-024-00993-6
PMID: 39881003
Abstract
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.
Details
- Title: Subtitle
- Hallmark discoveries in the biology of non-Wilms tumour childhood kidney cancers
- Creators
- Daniela Perotti - Fondazione IRCCS Istituto Nazionale dei TumoriMaureen J O'Sullivan - Trinity College DublinAmy L Walz - Northwestern UniversityJonathan Davick - University of IowaReem Al-Saadi - University College LondonDaniel J Benedetti - Vanderbilt University Medical CenterJack Brzezinski - Hospital for Sick ChildrenSara Ciceri - Fondazione IRCCS Istituto Nazionale dei TumoriNicholas G Cost - University of Colorado DenverJeffrey S Dome - George Washington UniversityJarno Drost - Princess Máxima CenterNicholas Evageliou - Children's Hospital of PhiladelphiaRhoikos Furtwängler - Saarland UniversityNorbert Graf - Saarland UniversityMariana Maschietto - Centro Infantil BoldriniElizabeth A Mullen - Dana-Farber/Boston Children's Cancer and Blood Disorders CenterAndrew J Murphy - St. Jude Children's Research HospitalMichael V Ortiz - Memorial Sloan Kettering Cancer CenterJustine N van der Beek - University Medical Center UtrechtArnauld Verschuur - Hôpital de la TimoneJenny Wegert - University of WürzburgRichard Williams - Imperial College LondonFilippo Spreafico - Fondazione IRCCS Istituto Nazionale dei TumoriJames I Geller - Cincinnati Children's Hospital Medical CenterMarry M van den Heuvel-Eibrink - University Medical Center UtrechtAndrew L Hong - Emory University
- Resource Type
- Journal article
- Publication Details
- Nature reviews. Urology, Vol.22(8), pp.526-543
- DOI
- 10.1038/s41585-024-00993-6
- PMID
- 39881003
- NLM abbreviation
- Nat Rev Urol
- ISSN
- 1759-4820
- eISSN
- 1759-4820
- Publisher
- NATURE PORTFOLIO
- Language
- English
- Electronic publication date
- 01/29/2025
- Date published
- 08/2025
- Academic Unit
- Pathology
- Record Identifier
- 9984781275102771
Metrics
2 Record Views