Standard Treatment Options
Treatment Options Under Clinical Evaluation

Because of the relative rarity of this tumor, all patients with rhabdoid tumor of the kidney 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.

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%, stage II of 47%, stage III of 22%, and stage IV of 8%.[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 (COG-Q9401).[2,3] 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 Children's Oncology Group clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

• COG-AREN0321: In this trial, 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.

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. 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]
   
   
3. 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]
   
   

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