Current Clinical Trials

The treatment of children with bilateral Wilms tumor must be individualized. The goals of therapy are to eradicate all tumor and to preserve as much normal renal tissue as possible with the hope of decreasing the risk of chronic renal failure among these children.[1,2] Studies demonstrate no difference in survival for children who undergo initial bilateral biopsy followed by chemotherapy and then surgical resection compared with patients who have initial resection followed by chemotherapy. Initially, patients should undergo bilateral renal biopsies with staging of each kidney. Primary tumor excision should not be attempted, but patients should be given preoperative chemotherapy. Initial treatment is with vincristine and dactinomycin (NWTS Regimen EE-4A) if the renal tumors are of favorable histology and not more extensive than stage II. Those with higher stage and favorable histology disease should receive doxorubicin, vincristine, and dactinomycin (NWTS Regimen DD-4A), and those with anaplastic histology should receive cyclophosphamide in addition to vincristine, doxorubicin, and etoposide (NWTS Regimen I). Following 6 weeks of chemotherapy, the patient should be reassessed. If serial imaging studies show no further reduction in tumor, a second-look surgical procedure should be performed (partial nephrectomy or wedge excision) if negative margins can be obtained; otherwise, sequential biopsies should be done to establish the reason for failure to respond. This approach will identify patients with anaplasia or differentiation, select them for early surgery, and define the intensity of chemotherapy to be administered.[3,4] Chemotherapy and/or radiation therapy following the second-look operation is dependent on the response to initial therapy, with more aggressive therapy required for patients with inadequate response to initial therapy observed at the second procedure.[3,5-9]

Renal transplantation for children with Wilms tumor is usually delayed until 1 to 2 years have passed without evidence of malignancy.[10] Similarly, renal transplantation for children with Denys-Drash syndrome and Wilms tumor, all of whom require bilateral nephrectomy, is generally delayed 1 to 2 years after completion of treatment for the tumor.[10]

Approximately 10% of patients with bilateral tumors have anaplastic histology and may benefit from more aggressive chemotherapy and radiation therapy and an aggressive surgical approach at the second-look operation.[11]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage V 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. Montgomery BT, Kelalis PP, Blute ML, et al.: Extended followup of bilateral Wilms tumor: results of the National Wilms Tumor Study. J Urol 146 (2 ( Pt 2)): 514-8, 1991.  [PUBMED Abstract]
   
   
2. Breslow NE, Takashima JR, Ritchey ML, et al.: Renal failure in the Denys-Drash and Wilms' tumor-aniridia syndromes. Cancer Res 60 (15): 4030-2, 2000.  [PUBMED Abstract]
   
   
3. Fuchs J, Wünsch L, Flemming P, et al.: Nephron-sparing surgery in synchronous bilateral Wilms' tumors. J Pediatr Surg 34 (10): 1505-9, 1999.  [PUBMED Abstract]
   
   
4. Shamberger RC, Haase GM, Argani P, et al.: Bilateral Wilms' tumors with progressive or nonresponsive disease. J Pediatr Surg 41 (4): 652-7; discussion 652-7, 2006.  [PUBMED Abstract]
   
   
5. Ritchey ML, Green DM, Thomas PR, et al.: Renal failure in Wilms' tumor patients: a report from the National Wilms' Tumor Study Group. Med Pediatr Oncol 26 (2): 75-80, 1996.  [PUBMED Abstract]
   
   
6. Horwitz JR, Ritchey ML, Moksness J, et al.: Renal salvage procedures in patients with synchronous bilateral Wilms' tumors: a report from the National Wilms' Tumor Study Group. J Pediatr Surg 31 (8): 1020-5, 1996.  [PUBMED Abstract]
   
   
7. Wilms' tumor: status report, 1990. By the National Wilms' Tumor Study Committee. J Clin Oncol 9 (5): 877-87, 1991.  [PUBMED Abstract]
   
   
8. Ritchey ML: The role of preoperative chemotherapy for Wilms' tumor: the NWTSG perspective. National Wilms' Tumor Study Group. Semin Urol Oncol 17 (1): 21-7, 1999.  [PUBMED Abstract]
   
   
9. Zuppan CW, Beckwith JB, Weeks DA, et al.: The effect of preoperative therapy on the histologic features of Wilms' tumor. An analysis of cases from the Third National Wilms' Tumor Study. Cancer 68 (2): 385-94, 1991.  [PUBMED Abstract]
   
   
10. Kist-van Holthe JE, Ho PL, Stablein D, et al.: Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant 9 (3): 305-10, 2005.  [PUBMED Abstract]
    
    
11. 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]
    
    

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