Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

Because of the relative rarity of this tumor, all patients with clear cell sarcoma of the kidney (CCSK) should be considered for entry into a clinical trial. Treatment planning by a multidisciplinary team of cancer specialists (pediatric surgeon or pediatric urologist, pediatric radiation oncologist, and pediatric oncologist) with experience treating renal tumors is required to determine and implement optimum treatment.

For CCSK, 5-year event-free survival (EFS) and overall survival (OS) for stage I is 100%; stage II is 87% and 97%, respectively; stage III is 74% and 87%, respectively; and stage IV is 36% and 45%, respectively (regimen I [see description below]).[1]

The approach for treating CCSK is different from Wilms tumor since the 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.[2] 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 (88% vs. 61% at 8 years).[3] 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 was approximately 89%, and OS was approximately 79%. Stage I patients had 100% 5-year EFS and OS. Stage II patients had a 5-year EFS of approximately 87% and OS of approximately 97%. Stage III patients had an approximately 74% 5-year EFS and an approximately 87% 5-year OS. Stage IV patients had a 5-year EFS of approximately 36% 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 was the brain,[1] which has been successfully treated with combination chemotherapy, surgery, and radiation therapy.[4][Level of evidence: 2A]

• Regimen DD-4A (vincristine, dactinomycin, and doxorubicin) for 15 months and radiation therapy.[3]



• Regimen I (vincristine, doxorubicin, cyclophosphamide, and etoposide) and radiation therapy.[1]

The following treatment option is currently under investigation in a Children's Oncology Group (COG) clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

• COG-AREN0321: In this trial, the role of radiation therapy is being evaluated in stage I disease. Patients who have undergone lymph node dissection will be treated only with regimen I. Patients with stage II and III will be treated with regimen I and radiation therapy. Stage IV 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.

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. 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. 
   
   
2. 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]
   
   
3. 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]
   
   
4. Radulescu VC, Gerrard M, Moertel C, et al.: Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer 50 (2): 246-9, 2008.  [PUBMED Abstract]
   
   

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