Anjum Nishan, Neha Madan


Clear cell sarcoma of the kidney is a rare malignant tumor mostly seen in children less than five years of age with a slight male preponderance. Patients usually present with a palpable abdominal lump, abdominal distention, pain and gross hematuria. It is an aggressive tumor with about 17-40% of patients presenting with bone metastasis. It is one of the renal tumor in children which is most frequently misdiagnosed as it is rare, has varied histomorphology and lack of specific diagnostic markers. However, BCOR gene have emerged as a recent molecular marker. Management includes total nephrectomy followed by postoperative chemotherapy and radiotherapy. We describe a case of clear cell sarcoma of kidney diagnosed on histopathology in a 2.5 year old male child.


Children, Clear cell sarcoma, Renal

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