Pediatric extrarenal malignant rhabdoid tumors (MRTs) are rare and aggressive (20% 5-year survival). Only 2 cases of bladder MRTs have been published. We report on a 9-year-old boy presenting with gross hematuria, palpable pelvic mass, and an obstructed, nonfunctional kidney. Evaluation was consistent with a 9.7 cm extrarenal MRT invading the bladder and prostate. He underwent a cystoprostatectomy, Indiana pouch continent urinary reservoir creation, and a left nephroureterectomy. A discrete 2.5 cm focus of renal MRT was found. To our knowledge, this is the first case of simultaneous perivesical and renal MRT. We review the current management of pediatric extrarenal MRTs.
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