Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Renal Cell Carcinoma
Standard Treatment Options
Survival of patients with renal cell carcinoma (RCC) 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, for stages II and III it is 50% to 80%, and for stage IV it is 9%, which is similar to the stage-for-stage survival in 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. In two small series, patients who had partial nephrectomies seemed to have outcomes equivalent to those who had radical nephrectomies. Partial nephrectomy may be considered in carefully selected patients with low-volume localized disease.[2,3] There is some suggestion that regional lymph node involvement does not portend the same poor prognosis as adult renal cell carcinoma. However, this is controversial as the finding are based on only 13 patients. 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. Rare spontaneous regression of pulmonary metastasis may occur with resection of the primary tumor. Several targeted agents (for example, sorafenib, sunitinib, bevacizumab, temsirolimus, pazopanib, and everolimus) have been approved for use in adults with RCC; however, these agents have not been tested in pediatric patients with RCC. However, a case report of an adolescent with a TFE-3 RCC suggests responsiveness to multiple tyrosine kinase inhibitors. (Refer to the PDQ summary on adult Renal Cell Cancer Treatment for more information.)
Treatment Options for Stages I and II
Hepatoblastoma of pure fetal histology: For tumors of pure fetal histology, complete surgical resection followed by watchful waiting or single-agent doxorubicin.In the Children's Oncology Group (COG) study COG-P9645, stage I pure fetal histology hepatoblastomas with two or fewer mitoses per 10 high power fields were not treated with chemotherapy. Completely excised tumor of purely fetal and favorable histology may be carefully followed without...
Check for U.S. clinical trials from NCI's list of cancer clinical trials 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.
Geller JI, Dome JS: Local lymph node involvement does not predict poor outcome in pediatric renal cell carcinoma. Cancer 101 (7): 1575-83, 2004.
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.
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.
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.
De Pasquale MD, Pessolano R, Boldrini R, et al.: Continuing response to subsequent treatment lines with tyrosine kinase inhibitors in an adolescent with metastatic renal cell carcinoma. J Pediatr Hematol Oncol 33 (5): e176-9, 2011.