Diagnostic Pathology (Aug 2011)

Review of juxtaglomerular cell tumor with focus on pathobiological aspect

  • Pan Chin-Chen,
  • Alvarado-Cabrero Isabel,
  • Petersson Fredrik,
  • Nagashima Yoji,
  • Inoue Keiji,
  • Mikami Shuji,
  • Ohe Chisato,
  • Gotoda Hiroko,
  • Kuroda Naoto,
  • Hes Ondrej,
  • Michal Michal,
  • Gatalica Zoran

DOI
https://doi.org/10.1186/1746-1596-6-80
Journal volume & issue
Vol. 6, no. 1
p. 80

Abstract

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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.