Clinical characteristics and outcomes of children with WAGR syndrome and Wilms tumor and/or nephroblastomatosis: The 30‐year SIOP‐RTSG experience

Background WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare contiguous gene deletion syndrome with a 45% to 60% risk of developing Wilms tumor (WT). Currently, surveillance and treatment recommendations are based on limited evidence. Methods...

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Veröffentlicht in:	Cancer 2021-02, Vol.127 (4), p.628-638
Hauptverfasser:	Hol, Janna A., Jongmans, Marjolijn C. J., Sudour‐Bonnange, Hélène, Ramírez‐Villar, Gema L., Chowdhury, Tanzina, Rechnitzer, Catherine, Pal, Niklas, Schleiermacher, Gudrun, Karow, Axel, Kuiper, Roland P., Camargo, Beatriz, Avcin, Simona, Redzic, Danka, Wachtel, Antonio, Segers, Heidi, Vujanic, Gordan M., Tinteren, Harm, Bergeron, Christophe, Pritchard‐Jones, Kathy, Graf, Norbert, Heuvel‐Eibrink, Marry M., Weid, Nicolas, Maricourt, Cécile Dumesnil, Proust, Stéphanie, Plantaz, Dominique, Perret, Cécile, Thomas, Caroline, Thebaud, Estelle, Boulanger, Cécile, Couteau, Nathalie, Verschuur, Arnauld C., Vaidya, Sucheta J., Oostveen, Radna Minou, Al‐Saadi, Reem, Maes, Philip, Werff‐ten Bosch, Jutte, Behrentz, Michael, Vujić, Dragana, Hasle, Henrik
Format:	Artikel
Sprache:	eng
Schlagworte:	Anaplasia - chemically induced Anaplasia - pathology Aniridia Anomalies Antineoplastic Protocols Child, Preschool Complications Confidence intervals Diagnosis Disease spread Drug-Related Side Effects and Adverse Reactions - epidemiology Drug-Related Side Effects and Adverse Reactions - pathology Female Gene Deletion Health services Hepatotoxicity Histology Humans Infant Kidney - drug effects Kidney - pathology Kidneys Liver - drug effects Liver - pathology Male Medicin och hälsovetenskap Metastases Metastasis Monitoring Oncology Original Patients pediatric Pediatrics predisposition Progression-Free Survival Protocol (computers) Regression analysis Risk Factors Surveillance Survival Telemedicine Toxicity treatment Tumors WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) WAGR Syndrome - complications WAGR Syndrome - drug therapy WAGR Syndrome - genetics WAGR Syndrome - pathology Wilms tumor Wilms Tumor - complications Wilms Tumor - drug therapy Wilms Tumor - genetics Wilms Tumor - pathology
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