Abstract

Clear cell sarcoma of the kidney (CCSK) is very uncommon and it constitutes 2-5% of paediatric renal tumour. It is one of the unfavourable renal tumours with high rate of recurrence and metastasis to the brain, bone and lungs. We reported a rare case of clear cell sarcoma in a 22-month-old girl who died shortly after surgery despite receiving neo-adjuvant chemotherapy for nine weeks. Even though clear cell sarcoma of the kidney is very rare, it is a close differential of nephroblastoma but with different treatment regimen and prognosis, hence correct diagnosis is important to improve survival and prevent recurrence.

Key words: clear cell sarcoma, nephroblastoma, bone metastasizing tumour, kidney