Renal oncocytosis in a pediatric patient: Case report and review of the literature

• Renal oncocytosis is a rare condition.
• 12 yr old female presented with flank pain and several masses in her right kidney.
• Final pathology revealed multiple benign oncocytomas.
• A review of the literature of this rare condition was performed.

Renal oncocytosis is a rare condition in which the kidney develops numerous oncocytomas. We present a case of a 12-year-old female who presented with right-sided flank pain of one-year duration. Imaging revealed several masses in the right kidney. Tissue biopsy confirmed multiple benign oncocytomas. Due to the presence of multiple oncocytomas throughout the kidney, a radical nephrectomy was performed. Given the rarity of this condition, as well as its known association with von Hippel-Lindau disease and Birt-Hogg-Dube syndrome, genetic investigations were pursued but failed to identify any abnormalities. This patient remains well and disease free six years after surgery. A review of the literature of this rare condition was performed.