Shao Lei, Manalang Michelle, Cooley Linda
Department of Pathology, Children's Mercy Hospitals and Clinics, School of Medicine, University of Missouri-Kansas City, Kansas City, Missouri, USA.
Pediatr Blood Cancer. 2008 Feb;50(2):406-9. doi: 10.1002/pbc.21048.
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.
肾小球旁细胞瘤（JGCT）是儿科人群中极为罕见的肾脏肿瘤。它被认为是一种起源于肾脏肾小球旁器的良性肿瘤。JGCT具有特征性的临床病理特征，但其细胞遗传学特征尚不清楚。我们报告一例8岁女性JGCT病例，该患者表现为严重高血压、血清肾素水平升高，右肾有一个边界清晰的肿瘤。通过电子显微镜检查在肿瘤细胞的细胞质中发现了肾素原颗粒。荧光原位杂交显示X、6、9、11、15和21号染色体单体。
