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Wilms tumour | Nature Reviews Disease Primers
Description:
Wilms tumour (WT) is a childhood embryonal tumour that is paradigmatic of the intersection between disrupted organogenesis and tumorigenesis. Many WT genes play a critical (non-redundant) role in early nephrogenesis. Improving patient outcomes requires advances in understanding and targeting of the multiple genes and cellular control pathways now identified as active in WT development. Decades of clinical and basic research have helped to gradually optimize clinical care. Curative therapy is achievable in 90% of affected children, even those with disseminated disease, yet survival disparities within and between countries exist and deserve commitment to change. Updated epidemiological studies have also provided novel insights into global incidence variations. Introduction of biology-driven approaches to risk stratification and new drug development has been slower in WT than in other childhood tumours. Current prognostic classification for children with WT is grounded in clinical and pathological findings and in dedicated protocols on molecular alterations. Treatment includes conventional cytotoxic chemotherapy and surgery, and radiation therapy in some cases. Advanced imaging to capture tumour composition, optimizing irradiation techniques to reduce target volumes, and evaluation of newer surgical procedures are key areas for future research. Wilms tumour (WT) is the most common renal tumour in infants and young children. This Primer reviews the epidemiology, mechanisms, diagnosis and management of WT. In addition, the authors outline potential opportunities to translate novel biological targets to improve clinical outcomes.
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pubmed, google, scholar, wilms, tumor, cancer, cas, central, tumour, pediatr, renal, study, oncol, report, group, children, oncology, childhood, clin, blood, treatment, tumors, nat, nature, clinical, childrens, siop, syndrome, mutations, patients, pediatric, national, tumours, genet, international, rev, kidney, surg, disease, pritchardjones, research, eur, stage, health, med, analysis, article, risk, results, development,
Topics {✒️}
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Questions {❓}
- From Wilms to kidney tumors: which ones require a biopsy?
- Is Wilms tumor a candidate neoplasia for treatment with WNT/β-catenin pathway modulators?
- Is radiotherapy required in first-line treatment of stage I diffuse anaplastic Wilms tumor?
- Review of phase I and II trials for Wilms’ tumour–can we optimise the search for novel agents?
- Technical considerations for nephron-sparing surgery in children: what is needed to preserve renal units?
- Why should survivors of childhood renal tumor and others with only one kidney be denied the chance to play contact sports?
- Wilms tumor and trisomy 18: is there an association?
- Fr/today/online-analysis-map?
- Gov/ct2/show/NCT04322318?
- Org/guidelines/guidelines-detail?
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name:Department of Medical Oncology and Hematology, Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
name:Department of Paediatrics, IWK Health, Dalhousie University, Halifax, Canada
name:Department of Paediatric Haematology and Oncology, Rigshospitalet, Copenhagen, Denmark
name:Cancer Control Center, Osaka International Cancer Institute, Osaka, Japan
name:Department of Pathology, Sidra Medicine, Doha, Qatar
name:Cincinnati Childrenâs Hospital Medical Center, University of Cincinnati, Cincinnati, Cincinnati, USA
name:Theodor-Boveri-Institute, Developmental Biochemistry, and Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany
name:Research Center, Boldrini Childrenâs Hospital, Genetics and Molecular Biology, Institute of Biology, State University of Campinas, Campinas, Brazil
name:Wellcome Sanger Institute, Hinxton, UK
name:Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
name:Department of Paediatrics, University of Cambridge, Cambridge, UK
name:National Cancer Research Institute Childrenâs Group Consumer Representative, London, UK
name:Department of Child Health, School of Medicine and Dentistry, Kwame Nkrumah University of Science and Technology, Kumasi, Ghana
name:Division of Hematology, Oncology and Bone Marrow Transplantation, Department of Paediatrics, University of Colorado, Aurora, USA
name:Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK
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