Clear cell variant of pediatric renal cell carcinoma: Rare and challenging.

We report a very rare case of clear cell variant of renal cell carcinoma (RCC) in a 3-year-old male child, who presented to us with a left-sided lump in his abdomen. Computed tomography (CT) scan and technetium-99 Dimercapto succinic acid (DMSA) scan revealed a large left renal mass with compromised left renal function. Left-sided nephroureterectomy was done and histopathology demonstrated clear cell carcinoma, possibly translocation-associated RCC (miT family) staged as pT2NxMx. Postoperative CT scans of the thorax and whole abdomen showed secondaries in the lungs and liver. We discussed treatment options in a multidisciplinary tumor board but meanwhile the child succumbed to respiratory failure.