Adjuvant Chemotherapy for Wilms Tumor in Children with Hemihyperplasia or Beckwith-Wiedemann Syndrome After Initial Neoadjuvant Therapy
This protocol applies to children with a localized unilateral renal tumor (favorable histology Wilms tumor) who also carry a genetic predisposing condition — hemihyperplasia or Beckwith-Wiedemann syndrome. Following neoadjuvant chemotherapy and surgical resection, adjuvant therapy is selected based on response and pathologic findings.
The preceding phase used neoadjuvant chemotherapy — the EE4A regimen, or the VAD regimen when an upfront biopsy was performed — followed by nephron-sparing or total nephrectomy with regional lymph node sampling at week 6 if the tumor was resectable, or at week 12 after continued chemotherapy. This protocol governs the adjuvant phase after that surgery.
Post-surgery, adjuvant chemotherapy is selected based on the tumor's response at week 6 and its pathologic stage and histology — options range from continuation of the initial regimen (without radiotherapy) to risk-adapted escalation of the chemotherapy backbone, with radiotherapy considered for certain stage categories. The complete regimen selection, sequencing, and all dosing details are in the full protocol.
Neoadjuvant therapy with the EE4A regimen is recommended for children with a localized unilateral renal tumor and a predisposing condition.
If patients have a complete response at 6 weeks to regimen EE4A, then they continue EE4A and do not receive RT.
Switching to regimen DD4A is recommended for patients who are at increased risk, including those with (1) stage III FHWT without blastemal predominant histology; or (2) stage I FHWT with blastemal predominant histology.
Augmented therapy with regimen I is recommended for patients with blastemal predominant histology and stage II or III FHWT, because they are at the greatest risk.
RT is often given 10 to 14 days after surgery; the patient's age and other factors are considered when deciding about the timing of RT.
DOI: 10.6004/jnccn.2021.0037 View source ↗