Advances in the clinical management of high-risk Wilms tumors

Michael V. Ortiz, Christa Koenig, Amy E. Armstrong, Jesper Brok, Beatriz de Camargo, Annelies M.C. Mavinkurve-Groothuis, Thelma B.Velasquez Herrera, Rajkumar Venkatramani, Andrew D. Woods, Jeffrey S. Dome, Filippo Spreafico

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations


Outcomes are excellent for the majority of patients with Wilms tumors (WT). However, there remain WT subgroups for which the survival rate is approximately 50% or lower. Acknowledging that the composition of this high-risk group has changed over time reflecting improvements in therapy, we introduce the authors’ view of the historical and current approach to the classification and treatment of high-risk WT. For this review, we consider high-risk WT to include patients with newly diagnosed metastatic blastemal-type or diffuse anaplastic histology, those who relapse after having been initially treated with three or more different chemotherapeutics, or those who relapse more than once. In certain low- or low middle-income settings, socio-economic factors expand the definition of what constitutes a high-risk WT. As conventional therapies are inadequate to cure the majority of high-risk WT patients, advancement of laboratory and early-phase clinical investigations to identify active agents is urgently needed.

Original languageEnglish
Article numbere30342
JournalPediatric Blood and Cancer
Issue numberS2
StatePublished - May 2023


  • COG
  • SIOP
  • Wilms tumor
  • high risk
  • nephroblastoma
  • relapsed


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