This protocol addresses children with stage I or II favorable-histology Wilms tumor (FHWT) who are classified as low risk following surgical resection. Risk stratification in this population is informed by molecular marker testing performed at the time of diagnosis and surgery.
Among children with stage I or II FHWT managed with adjuvant therapy, the presence of combined loss of heterozygosity (LOH) at chromosomes 1p and 16q identifies a subset with meaningfully lower relapse-free survival compared to those without these markers. This combined molecular finding drives a specific treatment decision for affected patients.
For low-risk FHWT patients whose tumors express combined LOH 1p and 16q, augmented adjuvant therapy with regimen DD4A is recommended over standard adjuvant chemotherapy. The complete regimen structure — including agents, schedule, and sequencing — is detailed in the full protocol.
Children with FHWT at low risk after surgery can receive adjuvant therapy with regimen EE4A or switch to regimen DD4A.
When treated with adjuvant EE4A, children with stage I or II FHWT with combined LOH at 1p and 16q had a 4-year relapse-free survival of 74.9% versus 91.2% for those without these markers.
DD4A is recommended for children with low-risk tumors that express combined LOH 1p and 16q.
DOI: 10.6004/jnccn.2021.0037
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