Wilms tumor
ICD-10 C64.1 · ICD-11 2C90.Y&XH5QN3

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

This protocol covers the treatment of children presenting with a metastatic unilateral renal tumor of favorable histology (Wilms tumor) who also have a genetic predisposition condition — specifically hemihyperplasia or Beckwith-Wiedemann syndrome.

Clinical Scenario

Pediatric patients with a unilateral renal tumor that has metastasized, confirmed favorable histology, and an underlying predisposing condition such as hemihyperplasia or Beckwith-Wiedemann syndrome. The presence of a genetic predisposition condition in this metastatic setting shapes the recommended treatment approach.

Treatment Approach (Partial Overview)

Management involves neoadjuvant therapy with the VAD regimen administered before surgery. The complete protocol specifies the full schedule, response-guided surgical criteria, and all sequencing details.

Clinical Goals

Complete or partial response of the tumor at 6 weeks, making the tumor resectable.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.6004/jnccn.2021.0037

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