Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Patients with rhabdoid tumor of the kidney continue to have a poor prognosis with 4-year overall survival (OS) for stage I patients of 33.3%, stage II of 46.9%, stage III of 21.8%, and stage IV of 8.4%.[1]

• No satisfactory treatment has been developed for these children. The National Wilms Tumor Study-5 (NWTS-5) closed the treatment arm for rhabdoid tumor with cyclophosphamide, etoposide, and carboplatin because of poor outcome. Combinations of etoposide and cisplatin; etoposide and ifosfamide; and ifosfamide, carboplatin, and etoposide (ICE chemotherapy) have been used.[2-4] In a review of 142 patients from NWTS 1-5, stage and age are significant prognostic factors. Patients with stage I and stage II disease had an OS rate of 42%; higher stage was associated with a 16% OS. Infants less than 6 months of age at diagnosis demonstrated a 4-year OS of 9%, whereas OS in patients aged 2 years and older was 41%. All except one patient with a CNS lesion died.[1]

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] Patients with stage I, II, III, and IV (without measurable disease) will be treated with UH-1, which consists of cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks and radiation therapy. Patients with stage III and IV (with measurable disease) will be treated with window therapy consisting of vincristine and irinotecan followed by UH-2 (cyclophosphamide, carboplatin, and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide for 30 weeks and radiation therapy) if they respond to window therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with rhabdoid tumor 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. Tomlinson GE, Breslow NE, Dome J, et al.: Rhabdoid tumor of the kidney in the National Wilms' Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol 23 (30): 7641-5, 2005.  [PUBMED Abstract]
   
   
2. Green DM, Children's Oncology Group: Phase III Multimodality Therapy Based on Histology, Stage, Age, and Tumor Size in Children With Wilms' Tumor, Clear Cell Sarcoma of the Kidney, or Rhabdoid Tumors of the Kidney, COG-Q9401, Clinical trial, Completed.  [PDQ Clinical Trial]
   
   
3. Waldron PE, Rodgers BM, Kelly MD, et al.: Successful treatment of a patient with stage IV rhabdoid tumor of the kidney: case report and review. J Pediatr Hematol Oncol 21 (1): 53-7, 1999 Jan-Feb.  [PUBMED Abstract]
   
   
4. Wagner L, Hill DA, Fuller C, et al.: Treatment of metastatic rhabdoid tumor of the kidney. J Pediatr Hematol Oncol 24 (5): 385-8, 2002 Jun-Jul.  [PUBMED Abstract]
   
   
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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