ABSTRACT

Clear cell sarcoma of kidney is a rare pediatric malignity, which constitutes 4 % of primary renal neoplasms of childhood. It is frequently seen between ages of 2 months and 14 years, and has a high propensity to metastasize as well as a high relapse rate. Our patient who was 7 months old baby, presented with a palpable abdominal mass and on detailed histopathologic examination it was confirmed to be clear cell sarcoma of kidney. It is aimed to draw attention on rare frequency of that aggressive patterned renal clear cell sarcoma in this pediatric age group and making careful differential diagnosis with Wilms tumor which can be misdiagnosed due to higher frequency in this age and similar microscopic features.