Treatment of Anaplastic 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 standard treatment option for anaplastic sarcoma of the kidney.

In a series of 40 patients with anaplastic sarcoma of the kidney who had staging data available, 13 had stage I disease, 12 had stage II disease, 10 had stage III disease, and 5 had stage IV disease.[1]

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Most patients (38 of 46) received up-front chemotherapy, and 14 patients received up-front radiation therapy. The 2-year event-free survival (EFS) rate was 81.8% for patients with stage I and stage II disease, compared with 46.6% for patients with stage III and stage IV disease (P = .07). The 2-year overall survival (OS) rate was 88.9% for patients with stage I and stage II disease, compared with 70.0% for patients with stage III and stage IV disease (P = .20).

Chemotherapy was associated with improved EFS and OS (hazard ratios [HRs], 0.09 and 0.08, respectively).

Patients (including those with stage I and stage II disease) treated with chemotherapy and surgery had lower risks of recurrence than those who had surgery alone.

The most common treatment protocols are based on Ewing sarcoma– and anaplastic Wilms tumor–directed therapies. These protocols use combinations of vincristine, doxorubicin, cyclophosphamide, and etoposide with or without ifosfamide, dactinomycin, and carboplatin.

Up-front radiation therapy was used in 14 patients. Only two recurrences were reported.

Older age at diagnosis was associated with worse outcomes (EFS HR, 1.08; OS HR, 1.07). Age groups of 0 to 3 years and 4 to 9 years had similar 2-year EFS rates (80% and 84.6%, respectively) and OS rates (100% and 92.3%, respectively). Patients older than 9 years had lower 2-year EFS rates (54.2%) and OS rates (68.4%) (EFS, P < .01; OS, P = .07).

In the posttherapy setting, surveillance for PPB, thyroid tumors, and/or ovarian tumors should be considered. Surveillance strategies will depend on which germline DICER1 pathogenic variant is detected and whether mosaicism is present.[1,11]

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Preserved source evidence · Not medical advice