What is the management of Wilms tumor (Nephroblastoma)?

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Last updated: March 17, 2025View editorial policy

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From the FDA Drug Label

1 INDICATIONS AND USAGE 1.1 Wilms Tumor Dactinomycin for Injection is indicated for the treatment of adult and pediatric patients with Wilms tumor, as part of a multi-phase, combination chemotherapy regimen.

2 DOSAGE AND ADMINISTRATION 2. 1 Recommended Dosage for Wilms Tumor The recommended dose of dactinomycin for injection, as part of a multi-agent combination chemotherapy regimen, is 45 mcg/kg intravenously once every 3 to 6 weeks for up to 26 weeks.

The management of Wilms tumor (Nephroblastoma) involves the use of dactinomycin for injection as part of a multi-phase, combination chemotherapy regimen. The recommended dose is 45 mcg/kg intravenously once every 3 to 6 weeks for up to 26 weeks 1. Key points to consider include:

  • Combination chemotherapy regimen: Dactinomycin is used in combination with other chemotherapy agents.
  • Dosing schedule: The recommended dose is administered every 3 to 6 weeks for up to 26 weeks.
  • Administration: The dose is given intravenously.

From the Research

The management of Wilms tumor involves a multidisciplinary approach combining surgery, chemotherapy, and sometimes radiation therapy, with the most recent and highest quality study suggesting that current evidence supports the use of nephron-sparing surgery (NSS) and minimally invasive approaches in selected cases 2. The initial treatment typically begins with nephrectomy (surgical removal of the affected kidney) to remove the primary tumor, allowing for accurate staging and histological assessment.

  • For unilateral tumors, radical nephrectomy is standard, while bilateral tumors may require nephron-sparing surgery to preserve renal function.
  • Preoperative chemotherapy with vincristine and actinomycin-D may be used for large tumors to facilitate surgical resection. Following surgery, patients receive adjuvant chemotherapy based on tumor stage and histology.
  • Favorable histology tumors at lower stages typically receive vincristine and actinomycin-D for 18-24 weeks, while higher stages or unfavorable histology may require more intensive regimens adding doxorubicin, cyclophosphamide, etoposide, or carboplatin.
  • Radiation therapy is indicated for higher-stage disease (stage III and IV) and anaplastic histology. Regular follow-up includes physical examinations, renal function tests, chest imaging, and abdominal ultrasounds to monitor for recurrence. This comprehensive approach has significantly improved survival rates, with over 90% of children with favorable histology now achieving cure, as reported in previous studies 3, 4, 5, 6. Treatment decisions should be individualized based on tumor characteristics, patient age, and overall health status to maximize cure rates while minimizing long-term complications, with recent advances in tumor imaging technology and potentially prognostic biomarkers being considered in the management of Wilms tumor 2.

References

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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