Juxtaglomerular cell tumor

reninomareninoma (juxtaglomerular cell tumor)
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells, with less than 100 cases reported in literature.wikipedia
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Secondary hypertension

secondary
It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare.
The differential diagnosis of a renal tumor in a young patient with hypertension includes Juxtaglomerular cell tumor, Wilms' tumor, and renal cell carcinoma, all of which may produce renin.

Renin

HyperreninemiaRENblood plasma renin
This tumor typically secretes renin, hence the former name of reninoma. The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.
The differential diagnosis of kidney cancer in a young patient with hypertension includes juxtaglomerular cell tumor (reninoma), Wilms' tumor, and renal cell carcinoma, all of which may produce renin.

Juxtaglomerular cell

juxtaglomerular cellsgranular cellsjuxtaglomerular apparatus cells
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells, with less than 100 cases reported in literature.

Hyperaldosteronism

secondary hyperaldosteronismmineralocorticoid excessaldosteronism
By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin.
One cause is a juxtaglomerular cell tumor.

Kidney tumour

kidney tumorrenal tumorsolid renal mass
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells, with less than 100 cases reported in literature.

Hypertension

high blood pressurehypertensivearterial hypertension
It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. Clinically, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT.

Renin–angiotensin system

renin–angiotensin–aldosterone systemrenin-angiotensin-aldosterone systemrenin angiotensin system
By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin.

Hypokalemia

hypokalaemialow blood potassiumhypokalemic
By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin.

Mesenchymal stem cell

mesenchymal stem cellsmesenchymal cellmesenchymal
JCT is morphologically characterized by multiple foci malignant mesenchymal epithelioid cells with, often with admixed necrosis, and a perivascular growth pattern.

Necrosis

necroticnecrotizingnecrotic tissue
JCT is morphologically characterized by multiple foci malignant mesenchymal epithelioid cells with, often with admixed necrosis, and a perivascular growth pattern.

CD34

antigens, cd34CD34 antigenCD34+
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Actin

F-actinG-actinactin cytoskeleton
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Syndecan 1

CD138syndecan-1SDC1
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Vimentin

VIM
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Type IV collagen

collagen IVcollagen type IVType-IV collagen
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Cytokeratin

cytokeratinsCK5.6pancytokeratin
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

S100 protein

S100S100 proteinsnerve tissue protein S 100
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

CD117

c-KitKITproto-oncogene proteins c-kit
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Desmin

DESDesmin related myopathy
The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Gross examination

grossgross autopsygrossly
Clinically, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT.

Renal cell carcinoma

renal cell cancerrenal carcinomakidney cancer
JCTs have a variable appearance and have often being misdiagnosed as renal cell carcinoma s; dynamic computed tomography is helpful in the differential diagnosis.

CT scan

computed tomographyCTCT scans
JCTs have a variable appearance and have often being misdiagnosed as renal cell carcinoma s; dynamic computed tomography is helpful in the differential diagnosis.

Differential diagnosis

differential diagnosesdifferentiateddifferential
Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms.

Hemangiopericytoma

HaemangiopericytomaHPC
Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms.

Glomus tumor

glomus tumourglomangioma
Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms.