Advances in the clinical management of high-risk Wilms tumors

Pediatr Blood Cancer. 2023 Mar;70(3):e30153. doi: 10.1002/pbc.30153. Epub 2023 Jan 10.


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.

Publication types

  • Review
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Humans
  • Kidney Neoplasms* / pathology
  • Neoplasm Staging
  • Prognosis
  • Recurrence
  • Wilms Tumor* / pathology