Treatment Options Under Clinical Evaluation
Current Clinical Trials

Regardless of histology, all stage I Wilms tumor patients have an excellent prognosis with the same treatment.[1]

For favorable-histology tumors (the 4-year relapse-free survival rate is 92%, and the 4-year overall survival [OS] rate is 98%):[2]

• Nephrectomy with lymph node sampling and 18 weeks of chemotherapy with vincristine and pulse-intensive dactinomycin (NWTS Regimen EE-4A).

For focal or diffuse anaplastic tumors (the 4-year event-free survival rate is 69.5%, and the 4-year OS rate is 82.6%):[3] In the National Wilms Tumor Study Group-5 (NWTS-5) study, patients with stage I Wilms tumor with diffuse and focal anaplasia were managed with vincristine and dactinomycin based on the excellent outcomes for this patient group in previous studies.[4] The outcomes of these patient groups in the NWTS-5 study were not as favorable as in previous studies.[3]

• Nephrectomy with lymph node sampling and 18 weeks of chemotherapy with vincristine and pulse-intensive dactinomycin.

It may be possible to treat a subset of stage I Wilms tumor patients with surgery alone without chemotherapy. The Children’s Oncology Group is planning a large study to address this question. In the NWTS-5, infants with stage I/FH Wilms tumor who were younger than 24 months and whose nephrectomy specimen weighed less than 550 g underwent surgery only.[5] The study was designed with a stringent stopping rule (interim analysis of RFS ≤90%), which was exceeded, mandating the closure of the study. Most patients could be successfully salvaged with chemotherapy, however, with a 2-year OS rate of 100%.

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

Favorable Histology

• AREN0532:[6] In this study, all tumors will be stratified based on central pathology review and molecular analysis (loss of heterozygosity [LOH] at chromosomes 1p and 16q). Patients with LOH at 1p and 16q will be upstaged to receive treatment with DD-4A (dactinomycin, doxorubicin, and vincristine) for 24 weeks. Patients who are younger than 2 years and have Wilms tumors that weigh less than 550 g and who have a negative microscopic evaluation of lymph nodes are eligible for observation only. Other stage I patients will be treated with the standard therapy regimen EE-4A (dactinomycin and vincristine) for 18 weeks postnephrectomy.

Anaplastic (focal or diffuse) histology

• AREN0321:[7] In this study, patients with stage I will be treated with standard DD-4A (vincristine, dactinomycin, and doxorubicin) and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I Wilms tumor. 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, 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]
   
   
2. 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]
   
   
3. Dome JS, Cotton CA, Perlman EJ, et al.: Treatment of anaplastic histology Wilms' tumor: results from the fifth National Wilms' Tumor Study. J Clin Oncol 24 (15): 2352-8, 2006.  [PUBMED Abstract]
   
   
4. Green DM, Beckwith JB, Breslow NE, et al.: Treatment of children with stages II to IV anaplastic Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 12 (10): 2126-31, 1994.  [PUBMED Abstract]
   
   
5. Green DM, Breslow NE, Beckwith JB, et al.: Treatment with nephrectomy only for small, stage I/favorable histology Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 19 (17): 3719-24, 2001.  [PUBMED Abstract]
   
   
6. Fernandez CV, Children's Oncology Group: Phase III Study of Adjuvant Vincristine and Dactinomycin With or Without Doxorubicin Hydrochloride and Radiotherapy or Observation Only in Younger Patients Who are Undergoing Nephrectomy for Newly Diagnosed Stage I-III Favorable Histology Wilms' Tumor, COG-AREN0532, Clinical trial, Active.  [PDQ Clinical Trial]
   
   
7. 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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