Treatment of Recurrent Clear Cell Sarcoma of the Kidney

Patients with most forms of recurrent childhood cancer should consider enrolling in phase I and phase II clinical trials. Other treatment options for clear cell sarcoma of the kidney are discussed below.

Palliative care remains a central focus of management regardless of whether disease-directed therapy is pursued at the time of progression. This ensures that quality of life is maximized while attempting to reduce symptoms and stress related to the terminal illness.

Clear cell sarcoma of the kidney has been characterized by late relapses. However, in trials after 1992, most relapses occurred within 3 years, and the most common sites of recurrence were the brain and the lungs.[3,5,16] In a series of 37 patients with clear cell sarcoma of the kidney who relapsed, the 5-year EFS rate after relapse was 18%, and the OS rate after relapse was 26%.[5]

The optimal treatment of relapsed clear cell sarcoma of the kidney has not been established. Treatment of patients with recurrent clear cell sarcoma of the kidney depends on initial therapy and site of recurrence.

Treatment options for recurrent clear cell sarcoma of the kidney include the following:

Cyclophosphamide and carboplatin should be considered if not used initially. Patients with recurrent clear cell sarcoma of the kidney, in some cases involving the brain, have responded to treatment with ifosfamide, carboplatin, and etoposide (ICE), coupled with local control consisting of surgical resection, radiation therapy, or both.[5]; [4][Level of evidence B4]

The use of high-dose chemotherapy followed by hematopoietic stem cell transplant (HSCT) is undefined in patients with recurrent clear cell sarcoma of the kidney. A total of 24 patients with relapsed clear cell sarcoma of the kidney received high-dose chemotherapy followed by autologous HSCT. Of those patients, 12 (50%) were alive without disease after a median of 52 months. Patients who had already achieved a second complete remission were more likely to receive high-dose chemotherapy.[4,5,19]