Treatment of Wilms Tumor in Children with Hemihyperplasia or Beckwith-Wiedemann Syndrome

A child with a localized unilateral kidney tumor of favorable histology in the setting of a predisposing genetic condition — hemihyperplasia or Beckwith-Wiedemann syndrome. The presence of a predisposing condition is a key factor that shapes the initial management approach.

The standard strategy begins with neoadjuvant chemotherapy before definitive surgery. The specific regimen used depends on whether an upfront biopsy was performed prior to starting treatment.

The full regimen options, timing, surgical decision criteria, lymph node sampling requirements, and response-adapted algorithm are detailed in the structured protocol.

The primary clinical goal is to achieve a complete or partial tumor response at week 6, making the tumor resectable — and enabling nephron-sparing surgery where achievable.