Table 3. Overview of Wilms Tumor Standard Treatment by Stage
| Stage | Histology | 4 Year RFS or EFS | 4 Year OS | Treatment (see Table 2 for chemotherapy regimen descriptions) |
| Stage I [1,2,4] | FH <24 mo/tumor weight <550g | 85% | 98% | Surgery only (should be done only within the context of a clinical trial) |
| FH >24 mo/tumor weight >550g | 94% RFS | 98% | Nephrectomy + lymph node sampling followed by regimen EE-4A | |
| DA | 68% EFS | 79%; (n = 10) | Nephrectomy + lymph node sampling followed by regimen EE-4A and XRT | |
| Stage II [1,2] | FH | 86% RFS | 98% | Nephrectomy + lymph node sampling followed by regimen EE-4A |
| FA | 80% EFS | 80%; (n = 5) | Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A | |
| DA | 83% EFS | 82% | Nephrectomy + lymph node sampling followed by abdominal XRT and regimen I | |
| Stage III [1,2] | FH | 87% RFS | 94% | Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A |
| FA | 88% RFS | 100%; (n = 8) | Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A | |
| FA (preoperative treatment) | 71% RFS | 71%; (n = 7) | Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal XRT | |
| DA | 46% EFS | 53%; (n = 16) | Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling and abdominal XRT | |
| DA | 65% EFS | 67% | Immediate nephrectomy + lymph node sampling followed by abdominal XRT and regimen I | |
| Stage IV [1,2] | FH | 76% RFS | 86% | Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A |
| FA | 61% EFS | 72%; (n = 11) | Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A | |
| DA | 33% EFS | 33%; (n = 15) | Immediate nephrectomy + lymph node sampling followed by abdominal XRT,a whole-lung XRT,b and regimen I | |
| DA (preoperative treatment) | 31% EFS | 44%; (n = 13) | Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling, followed by abdominal XRT,a and whole-lung XRTb | |
| Stage V [1-3] | Overall | 61% EFS | 80%; (n = 158) | |
| FH | 65% | 87% (4-yr OS); 78% (10-yr OS)c | Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen EE-4A (if disease in both kidneys ≤ stage II) or regimen DD-4A (if disease in both kidneys > stage II), followed by second-look surgery and possibly more chemotherapy and/or XRTd | |
| FA | 76% | 88%; (n = 9) | Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen I, followed by second-look surgery and possibly more chemotherapy and/or XRTd | |
| DA | 25% | 42%; (n = 20) | Bilateral renal biopsies and staging of each kidney followed by preoperative treatment with regimen I, followed by second-look surgery and possibly more chemotherapy and/or XRT d |
| AH = anaplastic histology; DA = diffuse anaplastic; EFS = event-free survival; FA = focal anaplastic; FH = favorable histology; OS = overall survival; RFS = relapse-free survival; XRT = radiation therapy | ||||
| aAbdominal XRT is planned according to local stage of renal tumor. | ||||
| bPulmonary XRT is reserved for patients with chest x-ray evidence of pulmonary metastases. | ||||
| c90% of the relapses occurred by 3.8 years from diagnosis and 90% of the deaths occurred within 5.7 years from diagnosis.[3] | ||||
| dThis approach is changing as noted on the AREN0534 study. |
References
- Grundy PE, Breslow NE, Li S, et al.: Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms Tumor Study Group. J Clin Oncol 23 (29): 7312-21, 2005. [PUBMED Abstract]
- 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]
- Ehrlich PF: Bilateral Wilms' tumor: the need to improve outcomes. Expert Rev Anticancer Ther 9 (7): 963-73, 2009. [PUBMED Abstract]
- Shamberger RC, Anderson JR, Breslow NE, et al.: Long-term outcomes for infants with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5. Ann Surg 251 (3): 555-8, 2010. [PUBMED Abstract]
