Treatment of Stage III Favorable Histology Wilms Tumor in Children — Standard Risk

This protocol applies to pediatric patients with stage III favorable histology Wilms tumor (FHWT) who are classified as standard risk following initial risk assessment.

Clinical Scenario

A child presents with stage III favorable histology Wilms tumor. After initial evaluation, the patient is stratified as standard risk. Risk classification at diagnosis is the key determinant of which treatment pathway is indicated and whether escalation is required.

Treatment Approach

The protocol defines a structured chemotherapy regimen for standard-risk stage III FHWT. Where specific molecular risk factors are identified, the protocol addresses escalation to an augmented multi-agent approach. The complete agent selection, sequencing, and criteria for escalation are specified in the full protocol …

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References

DOI: 10.6004/jnccn.2021.0037

DD4A is recommended for patients with stage III FHWT classified as standard risk after the initial risk assessment.

Switching to augmented therapy with regimen M is recommended for patients with combined LOH of 1p and 16q who are at increased risk.

Regimen M consists of 9 doses of vincristine, 5 doses of dactinomycin, 5 doses of doxorubicin (cumulative dose 150 mg/m2), 4 courses of 5 daily doses of cyclophosphamide, and 4 courses of 5 daily doses of etoposide over 24 weeks.

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