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Treatment of adults with Wilms tumor
Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 15, 2025 · Captured 2026-09-09.
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As Wilms tumor rarely occurs in adults, there is no standard treatment protocol. Better results have been reported for adults when they are treated in pediatric trials.
A Wilms tumor in an adult represents a therapeutic emergency because of the tumor's rapid growth and because urologists and oncologists are more familiar with the indolent growth of renal cell carcinoma.
The NWTS Group reported the outcomes for adult patients with Wilms tumor from the NWTS-1, -2, and -3 trials.[195-197]
The 3-year OS rate for adults on the NWTS-1 trial was 24% (compared with 74% in children) and improved to a 5-year OS rate of 82.6% on the NWTS-3 trial, although the number of adult patients treated on each trial was 31 or fewer.
These data suggest that many adults with Wilms tumor, if treated appropriately, can expect to be cured, especially if the tumor has not spread and/or is completely resected.
For adults with refractory or recurrent disease, screening for potential therapeutic targets in the tumor should be considered.[198]
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The following recommendations from the renal tumor committees of the International Society of Paediatric Oncology (SIOP) and COG encourage a uniform approach to improve outcome for adults with Wilms tumor.[199]
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Consult with a pediatric oncologist who has experience with the treatment of Wilms tumor as soon as a histological diagnosis is suspected.
Avoid delaying the start of chemotherapy. Ideally, chemotherapy, and radiation therapy if necessary, should be started by day 14 postnephrectomy, although delaying the start until day 30 is acceptable.
Be alert for toxicity of vincristine (neurotoxicity) and dactinomycin (hepatic toxicity) in adults.
Register patients in pediatric renal tumor trials if studies are available and the patients are eligible.
In a series of 14 adult Wilms tumors that were evaluated by expanded targeted sequencing, 5 (36%) demonstrated BRAF V600E variants. These tumors contained areas that were morphologically identical to BRAF V600E–altered metanephric adenoma. All of the BRAF V600E–altered Wilms tumors in this cohort occurred in patients older than 30 years. Identifying a BRAF V600E variant has therapeutic significance because these patients may respond to therapy with BRAF/MEK inhibitors.[136] There was one report of a male (aged 51 years) who had a relapsed metastatic Wilms tumor that harbored a BRAF V600E variant. The patient was treated with the BRAF inhibitor dabrafenib, and they had a prolonged and dramatic response.[198]
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