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 tumors of the kidney continue to have a poor prognosis with 4-year overall survival (OS) rates of 42% for stages I and II (n = 40) and 16% for stages III, IV, and V (n = 102).
No satisfactory treatment has been developed for these children. The National Wilms Tumor Study (NWTS)-5 (COG-Q9401) closed the treatment arm for rhabdoid tumor with cyclophosphamide, etoposide, and carboplatin because poor outcome was observed. Combinations of etoposide and cisplatin; etoposide and ifosfamide; and ifosfamide, carboplatin, and etoposide (ICE chemotherapy) have been used (COG-Q9401).[3,4] In a review of 142 patients from NWTS-1 through NWTS-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 younger than 6 months 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 central nervous system lesion died.
The Société Internationale d'Oncologie Pédiatrique (SIOP) renal tumor group has noted that preoperative chemotherapy does not seem to translate into improved survival. Delays in surgery lead to worse survival compared with patients treated according to direct surgery strategies.
Treatment Options Under Clinical Evaluation
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 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients with stages 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.