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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 11/07/2008



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 (11/07/2008)






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Renal Cell Carcinoma

Standard Treatment Options
Current Clinical Trials



Standard Treatment Options

Survival is affected by stage of disease at presentation and the completeness of resection at radical nephrectomy. Overall survival rates range from 64% to 87%. The 5 year survival for stage I is 90% or higher, stages II and III is 50% to 80%, and stage IV is 9%, which is similar to the stage-for-stage survival in renal cell carcinoma (RCC) in adults. Retrospective analyses and the small number of patients involved place limitations on the level of evidence in the area of treatment. The primary treatment for RCC includes total surgical removal of the kidney and associated lymph nodes.[1] In two small series, patients who had partial nephrectomies seem to have outcomes equivalent to those who have radical nephrectomies. Partial nephrectomy may be considered in carefully selected patients with low–volume localized disease.[2,3] There is no evidence that adjuvant therapy is beneficial in children with lymph-node positive, nonmetastatic disease.[1] Treatment of unresectable metastatic disease is presently unsatisfactory, similar to adult RCC; it is poorly responsive to radiation and there is no effective chemotherapy regimen. Immunotherapy, such as interferon-alpha and interleukin-2, may have some effect on cancer control.[4] Rare spontaneous regression of pulmonary metastasis may occur with resection of the primary tumor. (Refer to the PDQ summary on adult Renal Cell Cancer Treatment for more information.)

Current Clinical Trials

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood renal cell carcinoma. 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. Geller JI, Dome JS: Local lymph node involvement does not predict poor outcome in pediatric renal cell carcinoma. Cancer 101 (7): 1575-83, 2004.  [PUBMED Abstract]

  2. Cook A, Lorenzo AJ, Salle JL, et al.: Pediatric renal cell carcinoma: single institution 25-year case series and initial experience with partial nephrectomy. J Urol 175 (4): 1456-60; discussion 1460, 2006.  [PUBMED Abstract]

  3. Ramphal R, Pappo A, Zielenska M, et al.: Pediatric renal cell carcinoma: clinical, pathologic, and molecular abnormalities associated with the members of the mit transcription factor family. Am J Clin Pathol 126 (3): 349-64, 2006.  [PUBMED Abstract]

  4. Fyfe G, Fisher RI, Rosenberg SA, et al.: Results of treatment of 255 patients with metastatic renal cell carcinoma who received high-dose recombinant interleukin-2 therapy. J Clin Oncol 13 (3): 688-96, 1995.  [PUBMED Abstract]

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