Approach to the patient: reninoma.

A reninoma is a functional tumour 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 that surgical curative resection is ideal. The current optimal imaging and biochemical workup of reninoma and management approach (ideally, tumour excision with subtotal renal resection) are described.