Stage I
Stage II
Stage III
Stage IV

Stage I

The following treatment options are currently under investigation in Children's Oncology Group (COG) clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

• AREN0532: In this study (COG-AREN0532), all tumors will be stratified based on central pathology review and molecular analysis (loss of heterozygosity [LOH] at chromosomes 1p and 16q). Patients with LOH at 1p and 16q will be upstaged to receive treatment with DD-4A (dactinomycin, doxorubicin, and vincristine) for 24 weeks. Patients who are younger than 2 years and have Wilms tumors that weigh less than 550 g and who have a negative microscopic evaluation of lymph nodes are eligible for observation only. Other stage I patients will be treated with the standard therapy regimen EE-4A (dactinomycin and vincristine) for 18 weeks postnephrectomy.

Anaplastic (Focal or Diffuse) Histology

• AREN0321: In this study (COG-AREN0321), patients with stage I will be treated with standard DD-4A (vincristine, dactinomycin, and doxorubicin) and radiation therapy.

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

• COG-AREN0532: In this study, all tumors will be stratified based on central pathology review and molecular analysis (loss of heterozygosity [LOH] at chromosomes 1p and 16q). Patients with LOH at 1p and 16q will be upstaged to receive treatment with DD-4A (dactinomycin, doxorubicin, and vincristine) for 24 weeks. Stage II patients without LOH will be treated with standard therapy EE-4A (dactinomycin and vincristine) for 18 weeks postnephrectomy.

Focal Anaplastic

• Patients with stage II will be treated with standard DD-4A (vincristine, dactinomycin, and doxorubicin) for 24 weeks and radiation therapy.

Diffuse Anaplastic

• COG-AREN0321: In this study, 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.

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

• COG-AREN0532: Patients in this study will be treated with standard therapy of DD-4A (vincristine, dactinomycin, and doxorubicin) for 24 weeks and radiation therapy. Patients who have loss of heterozygosity at chromosomes 1p and 16q will be moved to AREN0533 with regimen M (consisting of vincristine, dactinomycin, and doxorubicin alternating with cyclophosphamide and etoposide) for a total of 24 weeks and radiation therapy.

Focal Anaplastic

• COG-AREN0321: In this trial, patients with stage III will be treated with standard DD-4A (vincristine, dactinomycin, and doxorubicin) for 24 weeks and radiation therapy.

Diffuse Anaplastic

• COG-AREN0321: 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.

The following treatment options are currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site.

Favorable Histology

• COG-AREN0533: In this trial, patients with pulmonary metastases only (detected by chest computerized tomography scans) will start treatment with standard chemotherapy DD-4A (vincristine, dactinomycin, and doxorubicin) and undergo abdominal irradiation if local stage III. 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 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 Anaplastic

• COG-AREN0321: 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 Anaplastic (No Measurable Disease)

• COG-AREN0321: 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 Anaplastic (Measurable Disease)

• COG-AREN0321: 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.

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