Clear Cell Sarcoma of the Kidney Treatment & Management

Updated: Aug 19, 2021
  • Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Medical Care

The approach for treating clear cell sarcoma of the kidney (CCSK) is different from the approach for Wilms tumor because the overall survival of children with clear cell sarcoma of the kidney remains considerably lower than that of patients with favorable-histology Wilms tumor. In the third National Wilms Tumor Study (NWTS-3), the addition of doxorubicin to the combination of vincristine, dactinomycin, and radiation therapy resulted in an improvement in disease-free survival in patients with clear cell sarcoma of the kidney. [26]

NWTS-4 showed that patients treated with vincristine, doxorubicin, and dactinomycin for 15 months had an improved relapse-free survival rate compared with patients treated for 6 months (87.5% vs 60.6% at 8 y). [22] The overall survival has improved for patients with clear cell sarcoma of the kidney from NWTS-3 to NWTS-4 (83% vs 66.9% at 8 y). The 8-year relapse-free survival rate for localized clear cell sarcoma of the kidney stages I-III is 88%, but late relapses have been known to occur. In the NWTS-5 protocol, patients with all stages of CCSK are treated with the same regimen used in patients who have Wilms tumor with diffuse anaplasia (excluding stage I); [26] this treatment consists of a radical nephrectomy followed by radiotherapy and chemotherapy with cyclophosphamide, etoposide, vincristine, and doxorubicin for 24 weeks.

In the NWTSG series that was reviewed by Argani et al, a better prognosis was indicated in the subset of patients with clear cell sarcoma of the kidney that was characterized by stage I tumors in patients aged 2-4 years in whom no tumor necrosis was identified. [4]

In the completed Children's Oncology Group protocol (AREN0321), all patients with clear cell sarcoma of the kidney, except patients with stage IV, were treated as in NWTS-5 with regimen I. However, patients with stage I who undergo lymph node sampling did not undergo radiation therapy to the tumor bed. Any patient with stage I who did not have a lymph node sampling was upstaged to stage II. [20] Patients with stage IV were treated with irinotecan and vincristine in an upfront window approach before treatment with cyclophosphamide, etoposide, vincristine, and  doxorubicin.


Surgical Care

At presentation, radical nephrectomy is the initial treatment of choice if the lesion is resectable. If the size or extension of the lesion is in question, a biopsy is performed, and chemotherapy is administered, followed by surgical resection after a response has been obtained.



Radiation oncologist

Once the tumor has been resected, the tumor bed and any other sites of disease are irradiated.

Pediatric oncologist

Primary care physicians should consult with a pediatric oncologist to determine standard and investigational treatment protocols.


Diet and Activity


No special diet is required.


Activity recommendations are made on an individual basis.