This protocol covers children with localized bilateral renal tumors (favorable histology Wilms tumor), with or without a predisposing condition, following completion of neoadjuvant chemotherapy and nephron-sparing surgery.
Children with localized bilateral renal tumors, confirmed favorable histology Wilms tumor, with or without a predisposing condition. Neoadjuvant therapy with the VAD regimen is the guideline-recommended first step for this population prior to surgical resection.
The prior treatment phase — the VAD regimen — targeted complete tumor response by week 6 and sufficient reduction to allow resectable bilateral disease. Following nephron-sparing surgery (bilateral partial nephrectomy, or total nephrectomy with contralateral partial nephrectomy, with regional lymph node sampling), this protocol specifies the adjuvant chemotherapy phase based on surgical and pathological findings.
Following surgery, an adjuvant chemotherapy regimen is assigned according to disease stage and histological findings — including the presence or absence of blastemal predominant histology — with radiation therapy indicated selectively based on disease extent.
DOI: 10.6004/jnccn.2021.0037
Neoadjuvant therapy with the VAD regimen is recommended for children with localized bilateral renal tumors with or without a predisposing condition.
Switching to regimen EE4A is recommended for patients with stage I FHWT without blastemal predominant histology, those with stage II FHWT with complete necrosis, or those with a complete response at 6 weeks of neoadjuvant chemotherapy.
Switching to regimen DD4A is recommended for patients with (1) stage II or 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 stage II or III FHWT with blastemal predominant histology, because they are at greatest risk.
Patients with a complete response at 6 weeks of neoadjuvant chemotherapy do not need RT.
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