Ectopic aldosterone-producing tumor

Ectopic aldosterone-producing tumor is an extremely rare cause of primary hyperaldosteronism (also known as Conn syndrome when caused by adrenal adenomas) in which aldosterone is secreted by a tumor located outside the adrenal glands. Unlike the typical forms of primary hyperaldosteronism caused by adrenal adenomas or bilateral adrenal hyperplasia, these tumors arise in ectopic locations, most commonly in the ovaries, kidneys, or other extra-adrenal sites. The excess aldosterone production leads to the same clinical consequences as adrenal-based hyperaldosteronism, primarily affecting the cardiovascular and renal systems. Key clinical features include resistant hypertension (high blood pressure that is difficult to control with standard medications), hypokalemia (low potassium levels in the blood), metabolic alkalosis, muscle weakness, fatigue, and in some cases cardiac arrhythmias related to electrolyte imbalances. Patients may also experience polyuria (excessive urination) and polydipsia (excessive thirst) due to the effects of hypokalemia on kidney function. The diagnosis can be particularly challenging because standard imaging of the adrenal glands may appear normal, and adrenal vein sampling — the gold standard for lateralizing aldosterone production in typical primary hyperaldosteronism — may yield confusing results. Treatment primarily involves surgical resection of the ectopic aldosterone-producing tumor when it can be identified and localized. Advanced imaging techniques, including CT scanning, MRI, and in some cases functional imaging, are essential for tumor localization. Prior to surgery or in cases where surgery is not feasible, medical management with mineralocorticoid receptor antagonists such as spironolactone or eplerenone can help control blood pressure and correct hypokalemia. The prognosis is generally favorable when the tumor is successfully identified and completely removed, often resulting in resolution or significant improvement of hypertension and electrolyte abnormalities.

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