Review of juxtaglomerular cell tumor with focus on pathobiological aspect

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.

Juxtaglomerular cell tumor: A morphological, immunohistochemical and genetic study of six cases

“…Approximately 100 cases of JGCT have been reported to date [3][4][5], but only a few cases have been analyzed for chromosomal abnormalities [6][7][8]. Recently, virtual karyotyping using single nucleotide polymorphisms (SNP) mapping array has been applied as a practical ancillary study for the classification of renal epithelial tumors [9][10][11][12][13][14].…”

Reninoma: A case report

“…Using electron microscopy, a search is made for intracytoplasmic granules, renin reservoirs that can be detected by Bowie staining and periodic acid-Schiff. The positivity of renin in the cytoplasm confirms the diagnosis, but it can also be positive in Wilms tumor, clear cell carcinomas and oncocytomas [5].…”

“…Reninoma is (+) for actin, CD34 and CD117, negative for cytokeratin, desmin, protein S-100, HMB-45, chromogranin and synaptophysin [5]. Using electron microscopy, a search is made for intracytoplasmic granules, renin reservoirs that can be detected by Bowie staining and periodic acid-Schiff.…”

Juxtaglomerular Cell Tumor that was Preoperatively Diagnosed Using Selective Renal Venous Sampling

“…The incidence of this tumor is very low. JCTs primarily affect adolescents and young adults, with a peak prevalence in the second and third decades of life, and are twice as common in women (8,13). All of the reported JCTs have been benign tumors, except for two cases (4,14).…”

“…To date, approximately 110 cases of JCT have been reported in the English literature (2)(3)(4)(5)(6)(7)(8). Patients with JCT are clinically characterized by hypertension with hyperaldosteronism secondary to excessive renin secretion by the tumor.…”