90% of pediatric kidney cancers
Wilms Tumor (Nephroblastoma) is the most common primary renal malignancy in children, accounting for approximately . As of 2025–2026 , clinical guidelines from the National Comprehensive Cancer Network (NCCN) and the Children's Oncology Group (COG) emphasize a multimodal approach that has pushed the 5-year survival rate for favorable histology cases above 90% . 1. Clinical Presentation & Predisposition
Prevention and Screening
For Unilateral Favorable Histology (COG AREN0532/53 updates):
Introduction
Cure Rates
: Thanks to multidisciplinary care (surgery, chemo, and radiation), the cure rate now exceeds 90% for many patients.
Outcomes and Prognosis
- Preoperative chemo (SIOP) reduces surgical complications; immediate nephrectomy (COG) provides pristine staging.
- New high-risk markers: 1q gain, TP53 mutation, blastemal type post-chemo.
- Relapse is not hopeless – incorporate B7-H3 or checkpoint inhibitors in trials.
- Reduce doxorubicin in infants; use proton therapy.
- Every survivor gets cardiac and renal surveillance.
- Imaging:
Wilms Tumor — Informative Paper