Advances in the clinical management of high-risk Wilms tumors.

Ortiz, Michael V; Koenig, Christa; Armstrong, Amy E; Brok, Jesper; de Camargo, Beatriz; Mavinkurve-Groothuis, Annelies M C; Herrera, Thelma B Velasquez; Venkatramani, Rajkumar; Woods, Andrew D; Dome, Jeffrey S; Spreafico, Filippo (2023). Advances in the clinical management of high-risk Wilms tumors. Pediatric blood & cancer, 70(3), e30153. Wiley 10.1002/pbc.30153

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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.

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