Bladder Recurrence of Clear Cell Sarcoma of the Kidney Seven Years After Initial Presentation

Clear cell sarcoma of the kidney (CCSK) is the second most common pediatric renal malignancy after Wilms tumor. CCSK has the potential to metastasize to distant sites and was historically known as the bone metastasizing renal tumor. We report an exceedingly rare case of a bladder recurrence of CCSK. Our patient presented with gross hematuria 7 years after initial complete response. He was found to have a large sessile bladder tumor and underwent a partial cystectomy with right pelvic lymph node dissection. Final pathology was metastatic CCSK.