New approaches to risk stratification for Wilms tumor

Curr Opin Pediatr. 2021 Feb 1;33(1):40-48. doi: 10.1097/MOP.0000000000000988. Epub 2020 Dec 29.

Abstract

Purpose of review: The treatment of Wilms tumor is one of the great achievements in the field of oncology. One of the key success factors has been improved risk stratification, enabling augmentation or reduction of therapy depending on a patient's risk of relapse. This article highlights the evolution of clinical and biological prognostic markers that have been applied in the treatment of Wilms tumor.

Recent findings: Historically, tumor stage and histology were the sole determinants of Wilms tumor treatment. Recent clinical trials conducted by the Children's Oncology Group (COG) and the International Society of Pediatric Oncology (SIOP) Renal Tumor Study Group have expanded the menu of prognostic factors to include histologic and volumetric response to therapy and tumor-specific loss of heterozygosity (LOH) at chromosomes 1p and 16q. Augmentation of therapy has been able to overcome the adverse risk factors. An emerging prognostic marker is chromosome 1q gain, will be incorporated into future clinical trials.

Summary: The application of new clinical and biological prognostic factors has created unprecedented ability to tailor therapy for Wilms tumor, accompanied with improved outcomes. Current and future trials will continue to enhance precision medicine for Wilms tumor.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Child
  • Humans
  • Kidney Neoplasms* / diagnosis
  • Kidney Neoplasms* / genetics
  • Kidney Neoplasms* / therapy
  • Neoplasm Recurrence, Local / genetics
  • Neoplasm Recurrence, Local / therapy
  • Neoplasm Staging
  • Risk Assessment
  • Wilms Tumor* / diagnosis
  • Wilms Tumor* / genetics
  • Wilms Tumor* / therapy