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Review
. 2023 May;70 Suppl 2(Suppl 2):e30342.
doi: 10.1002/pbc.30342.

Advances in the clinical management of high-risk Wilms tumors

Michael V Ortiz  1 , Christa Koenig  2 , Amy E Armstrong  3 , Jesper Brok  4   5 , Beatriz de Camargo  6 , Annelies M C Mavinkurve-Groothuis  7 , Thelma B Velasquez Herrera  8 , Rajkumar Venkatramani  9 , Andrew D Woods  10 , Jeffrey S Dome  11 , Filippo Spreafico  12
Affiliations
Review

Advances in the clinical management of high-risk Wilms tumors

Michael V Ortiz et al. Pediatr Blood Cancer. 2023 May.

Abstract

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.

Keywords: COG; SIOP; Wilms tumor; high risk; nephroblastoma; relapsed.

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Conflict of interest statement

CONFLICT OF INTEREST STATEMENT

Michael Ortiz has previously served as an expert advisor for Guidepoint Global and has received research funds from Amgen, none of which is relevant to anything discussed in this manuscript. Amy Armstrong has previously served as a consultant for Springworks and EM Partners, none of which is relevant to the content of this manuscript. All other authors declare no competing interests.

References

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