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|
|
Table 2. Overview of Wilms Tumor Standard Treatment by Stage
|
Stage
|
Histology
|
RFS or EFS
|
OS
|
Treatment (see Table 1 for chemotherapy regimen definitions)
|
| Stage I [1-3] |
FH |
92% RFS |
98% |
Nephrectomy + lymph node sampling followed by regimen EE-4A |
| FA or DA |
69% EFS |
83% |
Nephrectomy + lymph node sampling followed by regimen EE-4A and XRT |
| Stage II [1,2,4] |
FH |
85% RFS |
96% |
Nephrectomy + lymph node sampling followed by regimen EE-4A |
| FA (very small numbers) |
80% EFS |
80% |
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 |
90% RFS |
95% |
Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A |
| FA |
88% RFS |
100% |
Nephrectomy + lymph node sampling followed by abdominal XRT and regimen DD-4A |
| FA |
71% RFS |
71% |
Preoperative treatment with regimen DD-4A followed by nephrectomy + lymph node sampling and abdominal XRT |
| DA |
46% EFS |
53% |
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 [1,2,4] |
FH |
80% RFS |
90% |
Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A |
| FA |
61% EFS |
72% |
Nephrectomy + lymph node sampling, followed by abdominal XRT,a bilateral pulmonary XRT,b and regimen DD-4A |
| DA |
33% EFS |
33% |
Immediate nephrectomy + lymph node sampling followed by abdominal XRT,a whole-lung XRT,a and regimen I |
| DA |
31% EFS |
44% |
Preoperative treatment with regimen I followed by nephrectomy + lymph node sampling, followed by abdominal XRT,a and whole-lung XRTb |
| Stage V [1,5-13] |
FH |
65% |
78% (10 year OS) |
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 XRT |
| AH |
44% |
55% |
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 |
| AH = anaplastic histology; DA = diffuse anaplastic; EFS = event-free survival; FA = focal anaplastic; FH = favorable histology; OS = overall survival; RFS = relapse-free survival; XRT = flank 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. |
References
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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]
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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]
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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]
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Green DM, Breslow NE, Beckwith JB, et al.: Effect of duration of treatment on treatment outcome and cost of treatment for Wilms' tumor: a report from the National Wilms' Tumor Study Group. J Clin Oncol 16 (12): 3744-51, 1998.
[PUBMED Abstract]
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Montgomery BT, Kelalis PP, Blute ML, et al.: Extended followup of bilateral Wilms tumor: results of the National Wilms Tumor Study. J Urol 146 (2 ( Pt 2)): 514-8, 1991.
[PUBMED Abstract]
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Zuppan CW, Beckwith JB, Weeks DA, et al.: The effect of preoperative therapy on the histologic features of Wilms' tumor. An analysis of cases from the Third National Wilms' Tumor Study. Cancer 68 (2): 385-94, 1991.
[PUBMED Abstract]
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