Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

For clear cell sarcomas of the kidney (CCSK), 5-year event-free survival (EFS) for stage I is 100%, stage II is 87%, stage III is 74%, and stage IV is 36%.[1,2]

The approach for treating CCSK is different from Wilms tumor since the overall survival (OS) of children with CCSK remains considerably lower than for patients with favorable histology Wilms tumor. In the National Wilms Tumor Study-3 (NWTS-3), the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival for patients with CCSK.[3] NWTS-4 showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival compared with patients treated for 6 months (87.5% vs. 60.6% at 8 years).[2] In the NWTS-5 study, children with stages I to IV CCSK were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed. With this treatment, the 5-year EFS is approximately 89%, and OS is approximately 79%. Stage I patients have 100% 5-year EFS and OS. Stage II patients have a 5-year EFS of approximately 87% and OS of approximately 97%. Stage III patients have an approximately 74% 5-year EFS and an approximately 87% 5-year OS. Stage IV patients have a 5-year EFS of approximately 35% and 5-year OS of 45%. CCSK has been characterized by late relapses; however, in NWTS-5, most relapses occurred within 3 years. In NWTS-5, the most common site of recurrence is the brain.[4]

• Regimen DD-4A (vincristine, dactinomycin, and doxorubicin) for 15 months and radiation therapy.[2]



• Regimen I (vincristine, doxorubicin, cyclophosphamide, and etoposide) and radiation therapy.

The following treatment option is currently under investigation in a national and/or institutional clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

• AREN0321:[5] In this trial, the role of XRT is being evaluated in Stage I disease. Patients who have undergone lymph node dissection will be treated only with Regimen I (vincristine, doxorubicin, and cyclophosphamide alternating with cyclophosphamide and etoposide). Patients with Stage II and III will be treated with Regimen I and radiation therapy. Stage IV patients will be treated with UH-1 consisting of cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with clear cell sarcoma of the kidney. 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. Green DM, Breslow NE, Beckwith JB, et al.: Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 16 (1): 237-45, 1998.  [PUBMED Abstract]
   
   
2. Seibel NL, Li S, Breslow NE, et al.: Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group. J Clin Oncol 22 (3): 468-73, 2004.  [PUBMED Abstract]
   
   
3. Argani P, Perlman EJ, Breslow NE, et al.: Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 24 (1): 4-18, 2000.  [PUBMED Abstract]
   
   
4. Seibel NL, Sun J, Anderson JR, et al.: Outcome of clear cell sarcoma of the kidney (CCSK) treated on the National Wilms Tumor Study-5 (NWTS). [Abstract] J Clin Oncol 24 (Suppl 18): A-9000, 502s, 2006. 
   
   
5. Dome JS, Children's Oncology Group: Phase II Study of Combination Chemotherapy, Radiotherapy, and/or Surgery in Patients With High-Risk Renal Tumors, COG-AREN0321, Clinical trial, Active.  [PDQ Clinical Trial]
   
   

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