Table 7. Overview of Standard Treatment Options for Stage IV Wilms Tumora

Histology4-Year RFS or EFS4-Year OSTreatmentb
FH (with isolated lung nodules)85% EFS96%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-4A80% EFS96%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-4A99% EFS95%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% EFS100%Nephrectomy + lymph node sampling, abdominal XRTc radiation to sites of metastases, and regimen M
FH with extrapulmonary metastases, with or without lung metastases 76% EFS89% 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
FA61% EFS72% (n = 11)Nephrectomy + lymph node sampling, followed by abdominal XRT,c radiation to sites of metastases, bilateral pulmonary XRT,d and regimen DD-4A
DA33% EFS33% (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% EFS70% (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

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.,[109] Dome et al.,[181] Dix et al.,[263] Dix et al.,[261] Daw et al.,[264] and Benedetti et al.[274]

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) study.

From: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

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PDQ Cancer Information Summaries [Internet].
Bethesda (MD): National Cancer Institute (US); 2002-.
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