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← Wilms Tumor and Other Childhood Kidney Tumors

HEALTH PROFESSIONAL · SOURCE READING

COG approach

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.

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Context: Wilms Tumor / Treatment of Wilms Tumor / Treatment option overview for Wilms tumor / Radiation therapy

Up-front surgery provides histological confirmation and tumor extent, providing the rationale for adjuvant therapy, including radiation therapy. Besides histology, postoperative risk factors for worse local control include: (1) incomplete resection, (2) positive margins, and (3) nodal involvement. Radiation therapy is not used in patients with stage I or stage II FH Wilms tumor. For patients with FH stage III Wilms tumor, flank or abdominal radiation therapy is used for treatment. In cases of unfavorable histology (focal or diffuse anaplasia), flank or abdominal radiation therapy is indicated for all patients. For more information, see Table 3.

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Results of NWTS (COG RTC) trials have shown the following:

Flank radiation therapy covers the tumor bed, involved nodal region, and entire adjacent vertebral bodies at 10.8 Gy in 1.8-Gy fractions. The dose of radiation therapy is based on the results of the NWTS-3 study in which there was no increase in abdominal relapse for stage III FH patients receiving 10 Gy versus 20 Gy with DD-4A chemotherapy.[268]

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Whole-abdominal radiation therapy is 10.5 Gy in 1.5-Gy fractions and is used to treat diffuse spill or peritoneal metastasis.[264]

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In the closed COG AREN0321 (NCT00335556) study, the radiation therapy dose to the tumor bed was 10.8 Gy in 1.8-Gy fractions, with the exception of patients with stage III diffuse anaplasia, where a dose of 19.8 Gy in 1.8-Gy fractions was used. This remains the current standard of treatment.[267]

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Results of the early NWTS studies (1 and 2) suggested that a radiation therapy delay of more than 10 days after surgery resulted in worse local control, particularly in unfavorable histology Wilms tumor.[269,270] However, no difference in local control was found if radiation therapy was delayed more than 10 days after surgery for patients with stages II to IV FH tumors treated on the NWTS-3 or NWTS-4 trials.[103] More recent data from the National Cancer Database confirmed improved survival in patients with nonmetastatic Wilms tumor who received adjuvant radiation therapy less than or equal to 14 days postoperatively.[265]

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Results from the NWTS-3 and NWTS-4 trials indicated that there was no survival benefit of whole-lung irradiation in the setting of lung metastases seen on CT scan only.[271] Current COG guidelines allow for omission of whole-lung irradiation in cases of FH disease without extrapulmonary metastases, loss of heterozygosity at 1p and 16q, and complete response at 6 weeks after vincristine, dactinomycin, and doxorubicin.[185] When whole-lung irradiation is given, a dose of 12 Gy in 1.5-Gy fractions is indicated for children older than 12 months and 10.5 Gy in 1.5-Gy fractions for patients younger than 12 months with pulmonary metastasis.

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Other sites of metastatic disease in Wilms tumor are uncommon and may include liver, extra-abdominal nodes, brain, and bone. In the COG AREN0533 (NCT00379340) study, the radiation therapy doses being used for patients younger than 16 years are 19.8 Gy in 1.8-Gy fractions to liver and gross residual nodes, 21.6 Gy in 1.8-Gy fractions to the whole brain with a boost of 10.8 Gy in 1.8-Gy fractions to gross metastatic disease in the brain, and 25.2 Gy in 14 fractions for bone metastasis. For patients older than 16 years, the radiation therapy dose to the whole brain and bone is increased to 30.6 Gy in 1.8-Gy fractions.

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Table 3. Radiation Therapy Regimens used in Children’s Oncology Group AREN0532, AREN0533, and AREN0321 Protocols
Local/Locoregional Disease
XRT = radiation therapy.
aRequires whole-abdominal XRT in 1.5 Gy daily fractions. Patients with diffuse unresectable peritoneal implants receive 21 Gy.
bWhole-lung irradiation is given in 1.5 Gy daily fractions.
cNot all patients receive radiation therapy.
dA boost is given for macroscopic disease.
Stage IStage IIStage IIIStage III (diffuse spill, peritoneal metastasis, preoperative rupture)a
Favorable histologyNo XRTNo XRT10.8 Gy10.5 Gy
Focal anaplasia10.8 Gy10.8 Gy10.8 Gy10.5 Gy
Diffuse anaplasia10.8 Gy10.8 Gy19.8 Gy10.5 Gy + 9 Gy boost
Metastatic Disease
Stage IV LungStage IV LiverStage IV BrainStage IV Bone
Favorable histology10.5 Gy for age <12 monthsb,c; 12 Gy for age >12 monthsb,c19.8 Gy +/- 5.4 to 10.8 Gy boostd21.6 Gy + 10.8 Gy boost for age <16 years; 30.6 Gy for age >16 years25.2 Gy for age <16 years; 30.6 Gy for age >16 years
Focal or diffuse anaplasia10.5 Gy for age <12 monthsb; 12 Gy for age >12 monthsb19.8 Gy +/- 5.4 to 10.8 Gy boostd21.6 Gy + 10.8 Gy boost for age <16 years; 30.6 Gy for age >16 years25.2 Gy for age <16 years; 30.6 Gy for age >16 years

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