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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 01/09/2009



Purpose of This PDQ summary






General Information






Cellular Classification






Stage Information






Treatment Option Overview






Standard Treatment Options for Wilms Tumor






Treatment Options Under Clinical Evaluation for Wilms Tumor






Clear Cell Sarcoma of the Kidney






Rhabdoid Tumor of the Kidney






Neuroepithelial Tumor of the Kidney






Mesoblastic Nephroma






Renal Cell Carcinoma






Recurrent Wilms Tumor and Other Childhood Kidney Tumors






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Changes to This Summary (01/09/2009)






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Treatment Options Under Clinical Evaluation for Wilms Tumor

Stage I
Stage II
Stage III
Stage IV
Current Clinical Trials



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

  • 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 regimen 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

  • COG-AREN0321: In this study, patients with stage I will be treated with standard regimen DD-4A and radiation therapy.
Stage II

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 (LOH at chromosomes 1p and 16q). Patients with LOH at 1p and 16q will be upstaged to receive treatment with regimen DD-4A. Stage II patients without LOH will be treated with standard therapy regimen EE-4A postnephrectomy.

Focal Anaplastic

  • Patients with stage II will be treated with standard regimen DD-4A 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.
Stage III

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, patients will be treated with standard therapy regimen DD-4A and radiation therapy. Patients who have LOH at chromosomes 1p and 16q will be moved to clinical trail COG-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 regimen DD-4A and radiation therapy.

Diffuse Anaplastic

  • COG-AREN0321: In this trial, patients will be treated with the UH-1 regimen and radiation therapy.
Stage IV

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 [CT] scans) will start treatment with standard chemotherapy regimen DD-4A and undergo abdominal irradiation if local stage III. Pulmonary metastases will be re-evaluated at 6 weeks with chest CT scan. Patients with complete resolution of pulmonary metastases will be considered rapid complete responders and will continue therapy with regimen DD-4A without any pulmonary radiation therapy. Patients who do not have a complete response (slow incomplete responders) will be switched to regimen M (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 an LOH 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 and radiation therapy.

Diffuse Anaplastic (No Measurable Disease)

  • COG-AREN0321: In this trial, patients will be treated with the UH-1 regimen 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 and radiation therapy.
Current Clinical Trials

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I Wilms tumor, stage II Wilms tumor, stage III Wilms tumor, stage IV Wilms tumor and recurrent Wilms tumor and other childhood kidney tumors. 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.

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