Clear cell sarcoma of the kidney in an adult. A case report

Clear cell sarcoma of the kidney was initially thought to be a variant of Wilms tumour with an unfavourable prognosis.

A 23 years-old woman presented with a right abdominal mass. CT scan revealed a solid 23 cm tumour of the right kidney. A right radical nephrectomy with retroperitoneal lymph node dissection was accomplished. The histological diagnosis was clear cell sarcoma. A combination chemotherapy regimen (cisplatin and doxorubicin) was performed on six cycles.

After two years, there was no evidence of tumour in the abdomen and thorax on CT Scan. The patient is being well.

Optimal treatment is unknown, and surgery, radiotherapy and chemotherapy are used alone but mostly in combination.