Clear Cell Sarcoma of Kidney : Clinical Features

Comments:
Clinical Features: Clear cell sarcoma of kidney (CCSK) is the 2nd most common pediatric malignant renal neoplasm and accounts for 4-6% of all renal neoplasms in this age group. The age range is 2 months to 14 years; mean age at diagnosis is 3 years. Rare congenital as well as adult cases have been reported. It is more common in males (M:F=2:1).

There is no association with renal dysplasia, nephrogenic rests, and familial or genetic cancer predisposition syndromes. Most patients present with a large unilateral abdominal mass, abdominal pain, and hematuria. Other symptoms include vomiting, decreased oral intake, fever, constipation, and hypertension. Bilateral cases are extremely rare. Patients often present with Stage II (37%) or Stage III (34%) disease. Some patients present with bone pain or pathologic fractures resulting from bone metastases. Due to this tendency, it has previously been referred to as the bone-metastasizing renal tumor of childhood.

The image shows classic pattern of CCSK consisting of nests of small uniform polygonal to ovoid cells with clear cytoplasm, separated by fibrovascular septae.