TriangleHEALTH PROFESSIONAL · SOURCE READING
Treatment of stage IV Wilms tumor
Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 15, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Context: Wilms Tumor / Treatment of Wilms Tumor
Table 8 provides an overview of the standard treatment options and survival data for patients with stage IV Wilms tumor, based on published results.
Source links and citations
| Histology | 4-Year RFS or EFS | 4-Year OS | Treatmentb |
|---|---|---|---|
| CR = complete response; DA = diffuse anaplasia; EFS = event-free survival; FA = focal anaplasia; FH = favorable histology; LOH = loss of heterozygosity; OS = overall survival; RFS = relapse-free survival; XRT = radiation therapy. | |||
| aSource: Grundy et al.,[110] Dome et al.,[183] Dix et al.,[266] Dix et al.,[264] Daw et al.,[267] Benedetti et al.,[279] and Armstrong et al.[276] | |||
| bFor chemotherapy regimen descriptions, see Table 2. | |||
| cAbdominal XRT is planned according to local stage of renal tumor. | |||
| dPulmonary XRT is reserved for patients with chest x-ray/chest computed tomography evidence of pulmonary metastases. | |||
| eFor more information, see the AREN0533 (NCT00379340) and AREN0321 (NCT00335556) studies. | |||
| FH (with isolated lung nodules) | 85% EFS | 96% | Nephrectomy + lymph node sampling, followed by abdominal XRT,c +/- bilateral pulmonary XRT,d and regimen DD-4A or regimen Me |
| FH (no LOH of 1p and 16q) with isolated lung nodules with CR to DD-4A | 80% EFS | 96% | Nephrectomy + lymph node sampling, followed by abdominal XRTc and regimen DD-4A |
| FH (no LOH of 1p and 16q) with isolated lung nodules with incomplete response to DD-4A | 99% EFS | 95% | Nephrectomy + lymph node sampling, followed by abdominal XRTc and bilateral pulmonary XRTd and regimen M |
| FH (LOH of 1p and 16q) with isolated lung nodules (n = 18) | 100% | 100% | Nephrectomy + lymph node sampling followed by abdominal XRTc and bilateral pulmonary XRTd and regimen M |
| FH (with LOH of 1p and/or 16q) (n = 20) | 95% EFS | 100% | Nephrectomy + lymph node sampling, abdominal XRTc radiation to sites of metastases, and regimen M |
| FH with extrapulmonary metastases, with or without lung metastases | 76% EFS | 89% | Nephrectomy + lymph node sampling followed by abdominal XRTc, regimen M, and local control of other metastatic sites; if lung metastases are present, bilateral pulmonary XRTd |
| FA | 80% EFS | 80% (n = 5) | Nephrectomy + lymph node sampling, followed by abdominal XRT,c radiation to sites of metastases, bilateral pulmonary XRT,d and regimen UH1/revised UH1 (AREN0321, consisting of vincristine, doxorubicin, cyclophosphamide, carboplatin, and etoposide) |
| DA | 33% EFS | 33% (n = 10) | Immediate nephrectomy + lymph node sampling followed by abdominal XRT,c radiation to sites of metastases, whole-lung XRT,d and regimen I |
| DA (preoperative treatment) | 60% EFS | 70% (n = 10) | Preoperative treatment with regimen UH2 followed by nephrectomy + lymph node sampling, followed by abdominal XRT,c radiation to sites of metastases, and whole-lung XRTd |
Source links and citations
Stage IV disease is defined by the presence of hematogenous metastases to the lung, liver, bone, brain, or other sites, with the lung being the most common site. The presence of liver metastases at diagnosis is not an independent adverse prognostic factor in patients with stage IV Wilms tumor.[239]
Source links and citations
Continue within this section
Studies cited in this source section
Source citation is not a determination that a study applies to you.
Publication references
Read the original reference and check its publication notices.
Preserved source evidence · Not medical advice