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Treatment of Ewing Sarcoma of the Kidney
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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There is no consensus for the optimal treatment of Ewing sarcoma of the kidney because data are lacking. The current treatment approach is extrapolated from the experience of treating Ewing sarcoma at other sites with multimodal therapy consisting of multiagent chemotherapy, surgery, and/or radiation therapy for local control. The MD Anderson Cancer Center reported their experience with 30 patients (median age, 30.5 years) with Ewing sarcoma of the kidney over a period of 23 years.[5]
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Twenty percent of the patients had tumors confined to the kidney, 23% had tumors extending locally beyond the kidney, and 57% had metastasis at diagnosis (lungs, liver, and bone). The 4-year EFS and OS rates were 54% and 85%, respectively, for patients without metastasis at diagnosis, compared with 35% and 47%, respectively, for patients with metastasis.
Patients with tumors confined to the kidney and treated with nephrectomy and adjuvant chemotherapy have favorable outcomes compared with patients who did not complete adjuvant therapy. Twelve of 13 patients with nonmetastatic disease who underwent upfront nephrectomy had a favorable outcome when adjuvant chemotherapy was used.
Presence of tumor thrombus in the renal vein and/or inferior vena cave was associated with worse EFS (P = .053).
Unfavorable prognostic factors were local tumor extension beyond the kidney, tumor thrombus, and distant metastasis.
The most commonly used chemotherapy was vincristine, cyclophosphamide, doxorubicin, ifosfamide, etoposide, cisplatin, irinotecan, and temozolomide. The most common initial chemotherapy used was vincristine, doxorubicin, and cyclophosphamide, either alone (23%) or in combination with ifosfamide and etoposide (23%). The second most common combination used was vincristine, doxorubicin, and ifosfamide.
Treatment according to Ewing sarcoma protocols should be considered.[1]
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For more information, see Ewing Sarcoma and Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue Treatment.
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Preserved source evidence · Not medical advice