Treatment Options Under Clinical Evaluation
Current Clinical Trials

For favorable-histology tumors (the 4-year relapse-free survival rate is 80%, and the 4-year overall survival [OS] rate is 90%):[1,2]

• Nephrectomy with lymph node sampling, abdominal radiation according to local stage of renal tumor, bilateral pulmonary radiation for patients with chest x-ray evidence of pulmonary metastases, and 24 weeks of chemotherapy with vincristine, doxorubicin, and pulse-intensive dactinomycin.

For focal anaplastic tumors (for patients receiving preoperative therapy: the 4-year event-free survival [EFS] rate is 61.3%, and the OS rate is 71.6%):[3]

• Nephrectomy with lymph node sampling, abdominal radiation according to local stage of renal tumor, bilateral pulmonary radiation for patients with chest x-ray evidence of pulmonary metastases, and 24 weeks of chemotherapy with vincristine, doxorubicin, and pulse-intensive dactinomycin.

For diffuse anaplastic tumors (the 4-year EFS and OS rates are 33.3%; for patients receiving preoperative chemotherapy: the 4-year EFS rate is 30.8%, and the OS rate is 44.0%):[3]

• Nephrectomy with lymph node sampling, abdominal radiation, whole-lung radiation for patients with chest x-ray evidence of pulmonary metastases, and 24 weeks of chemotherapy with vincristine, doxorubicin, etoposide, cyclophosphamide, and mesna.[3]

The following treatment options are currently under investigation in national and/or institutional clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable histology

• AREN0533:[4] In this trial, patients with pulmonary metastases only will start treatment with standard chemotherapy DD-4A (vincristine, dactinomycin, and doxorubicin). Pulmonary metastases will be re-evaluated at 6 weeks with chest computerized tomography scan. Patients with complete resolution of pulmonary metastases will be considered rapid complete responders and will continue therapy with DD-4A without any pulmonary radiation therapy. Patients who do not have a complete response (slow incomplete responders) will be switched to Regimen M (consisting of vincristine, dactinomycin, and doxorubicin alternating with cyclophosphamide and etoposide for a total of 24 weeks and radiation therapy) and undergo radiation therapy to their lungs. It is recommended that biopsies on residual pulmonary lesions be performed before radiation therapy is delivered.

  Patients with a loss of heterozygosity at chromosomes 1p and 16q will be treated with Regimen M with radiation therapy to all sites of disease. Patients with metastases outside or in addition to lung metastases will be treated with Regimen M and radiation therapy.

Focal anaplasia

• AREN0321:[5] In this trial, patients will be treated with the UH-1 regimen (cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks) and radiation therapy.

Diffuse anaplasia (no measurable disease)

• AREN0321:[5] In this trial, patients will be treated with the UH-1 regimen (cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks) and radiation therapy.

Diffuse anaplasia (with measurable disease)

• AREN0321:[5] In this trial, patients will be treated with window therapy consisting of vincristine and irinotecan for 12 weeks. If they respond to the window therapy, they will receive therapy consisting of UH-2 (cyclophosphamide, carboplatin, and etoposide; vincristine, doxorubicin, and cyclophosphamide; vincristine, irinotecan, and radiation therapy) for 30 weeks. Patients not responding to the window therapy would then be treated on UH-1, which consists of cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV Wilms tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Green DM, Breslow NE, Beckwith JB, et al.: Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 16 (1): 237-45, 1998.  [PUBMED Abstract]
   
   
2. Green DM, Breslow NE, Beckwith JB, et al.: Effect of duration of treatment on treatment outcome and cost of treatment for Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 16 (12): 3744-51, 1998.  [PUBMED Abstract]
   
   
3. Dome JS, Cotton CA, Perlman EJ, et al.: Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. J Clin Oncol 24 (15): 2352-8, 2006.  [PUBMED Abstract]
   
   
4. Seibel NL, Children's Oncology Group: Phase III Study of Combination Chemotherapy With or Without Radiotherapy in Patients With Newly Diagnosed Stage III or IV Favorable Histology Wilms' Tumor, COG-AREN0533, Clinical trial, Active.  [PDQ Clinical Trial]
   
   
5. Dome JS, Children's Oncology Group: Phase II Study of Combination Chemotherapy, Radiotherapy, and/or Surgery in Patients With High-Risk Renal Tumors, COG-AREN0321, Clinical trial, Active.  [PDQ Clinical Trial]
   
   

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