Treatment of Initially Unresectable Wilms Tumor in Children with Favorable Histology and No Genetic Predisposition
Clinical Scenario
This protocol addresses children presenting with a unilateral renal tumor that is not surgically resectable at initial evaluation, where histology is favorable and no underlying genetic predisposition condition is present.
Approach (partial overview)
Management starts with upfront biopsy to establish histological diagnosis and obtain molecular biomarkers, followed by a neoadjuvant chemotherapy regimen. The full structured sequence — including how tumor response guides subsequent surgical decision-making — is detailed in the complete protocol.
Primary Treatment Goal
The target is for the tumor to become resectable following the initial phase of neoadjuvant chemotherapy, enabling planned surgical resection.
References
- DOI: 10.6004/jnccn.2021.0037
- Neoadjuvant therapy with DD4A is recommended for children with unilateral renal tumors that are initially unresectable if there are no predisposing conditions.
- Upfront biopsy is recommended for all patients meeting the criteria for delayed resection, to determine histology, establish a diagnosis of WT, and obtain molecular biomarkers to guide therapy.
- At week 6 of DD4A, the tumor is reimaged and depending on the tumor response, patients have either nephrectomy with regional lymph node sampling or continue with DD4A.
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