Juxtaglomerular cell tumor in an 8-year-old girl

Abstract

Juxtaglomerular cell tumor (JGCT) is an extremely rare renal neoplasm in the pediatric population. It is considered a benign tumor arising from the juxtaglomerular apparatus of the kidney. JGCT has characteristic clinicopathologic features, but its cytogenetic features are unknown. We report a case of JGCT in an 8-year-old female who presented with severe hypertension, elevated serum renin level, and a well circumscribed tumor in the right kidney. Protogranules of renin was identified in the cytoplasm of the tumor cells by electron microscopic examination. Fluorescence in situ hybridization revealed monosomy of chromosomes X, 6, 9, 11, 15, and 21.