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Diagn Pathol. 2011 Aug 26;6:80. doi: 10.1186/1746-1596-6-80.

Review of juxtaglomerular cell tumor with focus on pathobiological aspect.

Author information

1
Department of Diagnostic Pathology, Kochi Red Cross Hospital, Shin-honmachi 2-13-51, Kochi City, Kochi 780-8562, Japan. kurochankochi@yahoo.co.jp

Abstract

Juxtaglomerular cell tumor (JGCT) generally affects adolescents and young adults. The patients experience symptoms related to hypertension and hypokalemia due to renin-secretion by the tumor. Grossly, the tumor is well circumscribed with fibrous capsule and the cut surface shows yellow or gray-tan color with frequent hemorrhage. Histologically, the tumor is composed of monotonous polygonal cells with entrapped normal tubules. Immunohistochemically, tumor cells exhibit a positive reactivity for renin, vimentin and CD34. Ultrastructurally, neoplastic cells contain rhomboid-shaped renin protogranules. Genetically, losses of chromosomes 9 and 11 were frequently observed. Clinically, the majority of tumors showed a benign course, but rare tumors with vascular invasion or metastasis were reported. JGCT is a curable cause of hypertensive disease if it is discovered early and surgically removed, but may cause a fatal outcome usually by a cerebrovascular attack or may cause fetal demise in pregnancy. Additionally, pathologists and urologists need to recognize that this neoplasm in most cases pursues a benign course, but aggressive forms may develop in some cases.

PMID:
21871063
PMCID:
PMC3173291
DOI:
10.1186/1746-1596-6-80
[Indexed for MEDLINE]
Free PMC Article

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