Anaplastic variant of clear cell sarcoma of the kidney: a rare case report

Clear cell sarcoma (CCSK) of the kidney is an uncommon but distinctive pediatric renal tumor with a characteristic histological pattern and marked propensity for bone metastasis. The rare anaplastic variant constitutes about 3% of cases of CCSK and carries an unfavorable prognosis, with increased tumor recurrence and resistant to chemotherapy. This variant show high frequency of p53 gene mutation and p53 over expression in comparison to the usual CCSK. We present a case of anaplastic variant of CCSK in an 10-year-old boy with both cytologic and histologic features, highlighting the importance of recognizing this rare entity.