Juxtaglomerular cell tumor
Encyclopedia
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is a rare kidney cancer
Kidney cancer
Kidney cancer is a type of cancer that starts in the cells in the kidney.The two most common types of kidney cancer are renal cell carcinoma and urothelial cell carcinoma of the renal pelvis...

 that typically secretes renin
Renin
Renin , also known as an angiotensinogenase, is an enzyme that participates in the body's renin-angiotensin system -- also known as the Renin-Angiotensin-Aldosterone Axis -- that mediates extracellular volume , and arterial vasoconstriction...

. It involves the juxtaglomerular cell
Juxtaglomerular cell
The juxtaglomerular cells are cells in the kidney that synthesize, store, and secrete the enzyme renin. They are specialized smooth muscle cells in the wall of the afferent arteriole that delivers blood to the glomerulus...

s. It often causes hypertension
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...

, in adults and children, although among causes of hypertension it is rare. Its malignant potential is uncertain.

History

Juxtaglomerular cell tumor was first described in 1967. Since then, under 100 case reports have been published. Karyotyping of a small number of these tumors revealed a common loss of chromosomes 9 and 11.

Pathology

By hypersecretion of renin, JCT causes hypertension and hypokalemia
Hypokalemia
Hypokalemia or hypokalaemia , also hypopotassemia or hypopotassaemia , refers to the condition in which the concentration of potassium in the blood is low...

. Both of these conditions may be corrected by surgical removal of the tumor.

Diagnosis

Hypertension combined with evidence of a kidney tumor via imaging or gross examination
Gross examination
Gross examination or "grossing" is the process by which pathology specimens are inspected with the bare eye to obtain diagnostic information, while being processed for further microscopic examination....

 suggest a JCT. Presence of renin granules in pathology specimens differentiates
Differential diagnosis
A differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...

 this tumor from renal hemangiopericytoma
Hemangiopericytoma
A hemangeopericytoma is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with an especially aggressive behavior.It was characterized in...

and other renal tumors.

Prognosis

JCT often is described as benign, however one case of metastasis has been reported, so its malignant potential is uncertain. In most cases the tumor is encapsulated.
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