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How is Wilms tumor treated?
Last reviewed: 04.07.2025
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Treatment of Wilms tumor depends on the stage and histological structure. Nephroblastoma is one of the first tumors in the treatment of which a comprehensive approach was used.
Treatment of Wilms' tumor (National Wilms' Tumor Study Group protocol)
Stage |
Treatment measures |
Stage I (favorable histologic structure) |
Surgery, no radiation therapy, chemotherapy - dactinomycin + vincristine |
Stage I anaplasia (unfavorable histological structure) |
Surgery, no radiation therapy, chemotherapy (dactinomycin + vincristine) for 6 months |
Stage II (favorable histology) |
Surgery, no radiation therapy, chemotherapy in regimen 1 (dactinomycin + vincristine) |
Stage III (favorable histology) |
Surgery, radiation therapy to the tumor bed in a total focal dose of 10.8 Gy, chemotherapy - dactinomycin + vincristine + Doxorubicin |
Stage IV (favorable histology) |
Surgery, radiation therapy to the tumor bed in a total focal dose of 10.8 Gy, chemotherapy - dactinomycin + vincristine + doxorubicin |
Stage II-IV (unfavorable histological structure) |
Cyclophosphamide is added to dactinomycin, vincristine and doxorubicin |
For children under 12 months, it is recommended to reduce the dose of all chemotherapy drugs by 50%. Chemotherapy treatment with full doses of drugs is carried out for children over 12 months.
Surgical treatment involves tumor removal, determination of its histological type and stage of the disease. Radiation therapy for nephroblastoma is used in some cases at stages III and IV of the disease. The chemotherapy regimen for nephroblastoma depends on the stage of the disease and includes the use of dactinomycin, vincristine and doxorubicin. Preoperative chemotherapy is rarely used in the USA, but it is mandatory in Europe. One of the problems with preoperative chemotherapy is the erroneous preoperative diagnosis of Wilms tumor in 6% of cases. The SIOP research group argues for the need for preoperative chemotherapy by the fact that most erroneous diagnoses are neuroblastoma. SIOP studies have shown that preoperative chemotherapy reduces the incidence of tumor rupture during surgery from 32% to 4%, and also reduces the stage of the disease - 80% of patients after 4 weeks of preoperative chemotherapy with vincristine and dactinomycin had stage I-II nephroblastoma at the time of surgery. In the United States, preoperative chemotherapy is used only in patients with disseminated disease or inoperable tumors, as well as in bilateral nephroblastoma.
Forecast
The prognosis of patients with nephroblastoma depends on a number of factors. Unfavorable prognostic factors:
- III-V stage;
- metastases to the para-aortic lymph nodes;
- anaplastic or sarcomatous histological structure;
- tumor rupture before or during surgery;
- liver metastases (metastasis to the lungs is more favorable than metastasis to the liver).
Wilms tumor treatment results
Stage (favorable histological structure) |
Two-year relapse-free survival, % |
Overall four-year survival rate, % |
I |
89 |
95.6 |
II |
87.4 |
91.1 |
III |
82 |
90.9 |
IV |
79 |
80.9 |
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