期刊
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
卷 -, 期 -, 页码 -出版社
ENDOCRINE SOC
DOI: 10.1210/clinem/dgad516
关键词
reninoma; juxtaglomerular cell tumor; secondary aldosteronism; secondary hypertension; renin
A reninoma is a functional tumor that hyperactivates the renin-angiotensin-aldosterone system, resulting in severe hypertension and hypokalemia. It is a potentially curable cause of secondary hyperaldosteronism, with a higher prevalence in women of childbearing age. Reninoma can be differentiated from renovascular hypertension and should be managed through surgical resection or with medications that are safe during pregnancy.
A reninoma is a functional tumor of afferent arteriolar juxtaglomerular cells that secretes the enzyme renin, leading to hyperactivation of the renin-angiotensin-aldosterone system. Reninoma is a potentially curable cause of pathological secondary hyperaldosteronism that results in often severe hypertension and hypokalemia. The lack of suppression of plasma renin contrasts sharply with the much more common primary aldosteronism, but diagnosis is often prompted by screening for that condition. The major differential diagnosis of reninoma is renovascular hypertension. Fewer than 200 cases of reninoma have been described. Reninomas have been reported across a broad demographic but have a 2:1 predilection for women, often of childbearing age. Aldosterone receptor blockade, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers offer effective medical management but are contraindicated in pregnancy, so surgical curative resection is ideal. The current optimal imaging and biochemical workup of reninoma and management approach (ideally, tumor excision with subtotal renal resection) are described.
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