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← Wilms Tumor and Other Childhood Kidney Tumors

HEALTH PROFESSIONAL · SOURCE READING

Surgery, chemotherapy, and radiation therapy

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: Clear Cell Sarcoma of the Kidney / Treatment of Clear Cell Sarcoma of the Kidney

Evidence (surgery, chemotherapy, and radiation therapy):

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

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In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

In the AREN0321 study, the 4-year EFS rate was 82.7%, and the OS rate was 95.1%.

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

For patients treated in both studies with the same stage-appropriate therapy, the 4-year EFS rates were 90.0% for stage I (n = 10), 93.4% for stage II (n = 47), 82.8% for stage III (n = 65), and 58.3% for stage IV (n = 10) patients. The 4-year OS rates were 100% for stage I, 97.9% for stage II, 93.8% for stage III, and 77.8% for stage IV patients.

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

No local recurrences were seen in patients with stage I disease who did not receive radiation therapy (n = 7). One patient with stage I disease experienced a brain recurrence.

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

Among patients with local stage III tumors, initial procedure type, margin status, and lymph node involvement were not prognostic factors.

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

The brain remains the most common site of relapse (n = 8). Fourteen relapses occurred within 3 years, including ten that occurred within 2 years. The latest relapse occurred at 3.5 years.

In the AREN0321 (NCT00335556) study, patients (n = 82) with stages I, II, and III disease were treated with regimen I, which consisted of vincristine, doxorubicin, cyclophosphamide, and etoposide. Based on the excellent results of stage I patients in the NWTS-5 study, radiation therapy was omitted for patients with stage I disease who had lymph nodes sampled. Patients with stage IV disease were treated with a carboplatin-containing regimen. There were 50 patients in the AREN03B2 (NCT00898365) study who were treated with the same stage-appropriate therapy. The results from the two studies are as follows:[16]

Patients with stage IV disease appeared to benefit from the addition of carboplatin.

In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

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In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

With this treatment, the 5-year EFS rate was 79%, and the OS rate was 90%.

In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

Stage I patients had 5-year EFS and OS rates of 100%.

In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

Stage II patients had a 5-year EFS rate of 88% and a 5-year OS rate of 98%.

In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

Stage III patients had a 5-year EFS rate of 73% and a 5-year OS rate of 89%.

In the National Wilms Tumor Study (NWTS) NWTS-5 (COG-Q9401/NCT00002611) trial, children with stages I to IV clear cell sarcoma of the kidney were treated with a new chemotherapeutic regimen combining vincristine, doxorubicin, cyclophosphamide, and etoposide in an attempt to further improve the survival of these high-risk groups. All patients received radiation therapy to the tumor bed.[3]

Stage IV patients had a 5-year EFS rate of 29% and a 5-year OS rate of 36%.

A review of patients with stage I clear cell sarcoma of the kidney treated on the NWTS-1, NWTS-2, NWTS-3, NWTS-4, and NWTS-5 trials showed an excellent OS rate of 100% with a wide variety of chemotherapy and radiation therapy regimens.[17]

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In the NWTS-3 trial, the addition of doxorubicin to the combination of vincristine, dactinomycin (DD-4A), and radiation therapy resulted in an improvement in disease-free survival for patients with clear cell sarcoma of the kidney.[1] The NWTS-4 trial expanded on this trial, with patients randomly assigned to either 15 months of therapy or 6 months of therapy.[18]

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In the NWTS-3 trial, the addition of doxorubicin to the combination of vincristine, dactinomycin (DD-4A), and radiation therapy resulted in an improvement in disease-free survival for patients with clear cell sarcoma of the kidney.[1] The NWTS-4 trial expanded on this trial, with patients randomly assigned to either 15 months of therapy or 6 months of therapy.[18]

Patients who were treated with DD-4A for 15 months had an improved relapse-free survival rate compared with patients who were treated for 6 months (88% vs. 61% at 8 years).

Studies cited in this source section

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Preserved source evidence · Not medical advice