{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T03:35:33Z","timestamp":1782790533194,"version":"3.54.5"},"reference-count":56,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"5","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["JCO Precis Oncol"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:sec>\n                    <jats:title>PURPOSE<\/jats:title>\n                    <jats:p>Molecular biomarkers are increasingly used for risk stratification, particularly in up-front surgery settings (Children's Oncology Group trials), whereas in preoperative chemotherapy setting, the ongoing International Society of Pediatric Oncology (SIOP)-Renal Tumor Study Group-2016 UMBRELLA study aims to validate selected biomarkers for future risk-adapted treatment strategies. This systematic review summarizes all literature on the prognostic value of these biomarkers.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>MATERIALS AND METHODS<\/jats:title>\n                    <jats:p>\n                      A systematic literature review (PubMed and Embase; up to January 2025) included studies with \u226550 de novo Wilms tumors (WTs). Eligible biomarkers included copy number variations; 1q gain, 1p and\/or 16q loss of heterozygosity (LOH)\/loss, 12 gain, 14q loss, 22 loss, 11p15 LOH\/loss of imprinting (LOI), and structural somatic variants (\n                      <jats:italic toggle=\"yes\">TP53<\/jats:italic>\n                      [and\/or 17p loss],\n                      <jats:italic toggle=\"yes\">MYCN<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">FBXW7<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">WT1<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">WTX<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">SIX1\/SIX2<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">DROSHA<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">DGCR8<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">AMER1<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">CTNNB1<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">GPC3<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">MLLT1<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">DICER1<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">DIS3L2<\/jats:italic>\n                      ). Outcome included relapse-free survival, event-free survival (EFS), and overall survival (OS). Risk of bias was assessed with quality in prognosis studies tool.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      Low-bias multivariable\/stratified analyses identified 1q gain as worse EFS and 1p and\/or 16q LOH\/loss as worse EFS\/OS prognostic factors, in up-front nephrectomy settings. Preoperative chemotherapy settings revealed similar trends with lacking significance.\n                      <jats:italic toggle=\"yes\">TP53<\/jats:italic>\n                      and\n                      <jats:italic toggle=\"yes\">MYCN<\/jats:italic>\n                      were adverse prognostic in univariate analyses. No prognostic data were available for the remaining variants.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSION<\/jats:title>\n                    <jats:p>\n                      1q gain and 1p and\/or 16q LOH\/loss emerge as independent prognostic biomarkers in up-front nephrectomy settings. Evidence remains limited in preoperative chemotherapy settings, particularly when using SIOP-oriented treatment algorithms. Prognostic value of\n                      <jats:italic toggle=\"yes\">TP53<\/jats:italic>\n                      ,\n                      <jats:italic toggle=\"yes\">MYCN<\/jats:italic>\n                      , and 11p15 LOH\/LOI warrants further validation in both settings. This highlights the need for adequately powered prospective studies, specifically in the preoperative chemotherapy setting, to establish reliable molecular biomarkers.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1200\/po-25-01017","type":"journal-article","created":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T19:59:14Z","timestamp":1779134354000},"update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":1,"title":["Systematic Review: Prognostic Molecular Biomarkers in Wilms Tumors"],"prefix":"10.1200","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8183-3167","authenticated-orcid":true,"given":"Agustina","family":"Oller","sequence":"first","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2237-7354","authenticated-orcid":true,"given":"Patrick","family":"Kemmeren","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology, Utrecht, the Netherlands"},{"name":"Center for Molecular Medicine, UMC Utrecht and Utrecht University, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6703-2889","authenticated-orcid":true,"given":"Daniela","family":"Perotti","sequence":"additional","affiliation":[{"name":"Predictive Medicine: Molecular Bases of Genetic Risk, Experimental Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4626-8702","authenticated-orcid":true,"given":"Harm","family":"van Tinteren","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1070-4442","authenticated-orcid":true,"given":"Arnauld","family":"Verschuur","sequence":"additional","affiliation":[{"name":"Department of Pediatric Haematology-Oncology, La Timone Children's Hospital, AP-HM, Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5587-3509","authenticated-orcid":true,"given":"Filippo","family":"Spreafico","sequence":"additional","affiliation":[{"name":"Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy"},{"name":"Oncology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesper","family":"Brok","sequence":"additional","affiliation":[{"name":"Department of Paediatric Oncology and Haematology, Rigshospitalet, Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1967-8343","authenticated-orcid":true,"given":"Rhoikos C.J.","family":"Furtw\u00e4ngler","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology and Oncology, Department of Pediatrics, Inselspital University Hospital, University of Bern, Bern, Switzerland"},{"name":"Department of Pediatric Hematology and Oncology, Saarland University, Homburg, Saarland, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanzina","family":"Chowdhury","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reem","family":"Al-Saadi","sequence":"additional","affiliation":[{"name":"Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, United Kingdom"},{"name":"Department of Histopathology, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0726-6939","authenticated-orcid":true,"given":"Gordan M.","family":"Vujanic","sequence":"additional","affiliation":[{"name":"Department of Pathology, Sidra Medicine, Doha, Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy L.","family":"Treece","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Children's of Alabama, Birmingham, AL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2941-6179","authenticated-orcid":true,"given":"Jarno","family":"Drost","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology, Utrecht, the Netherlands"},{"name":"Oncode Institute, Utrecht, the Netherlands"},{"name":"Division Cell Biology, Metabolism &amp; 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