Adrenocortical carcinoma with pure aldosterone hypersecretion

Adrenocortical carcinoma with pure aldosterone hypersecretion is an extremely rare malignant tumor of the adrenal cortex (Orphanet code 231625; ICD-10 C74.0) that exclusively produces excess aldosterone without significant co-secretion of other adrenal hormones such as cortisol or androgens. This distinguishes it from more common functioning adrenocortical carcinomas, which typically secrete multiple hormones. The condition primarily affects the endocrine and cardiovascular systems. Excess aldosterone leads to a clinical picture of primary hyperaldosteronism (Conn syndrome), characterized by severe or treatment-resistant hypertension, hypokalemia (low potassium levels), metabolic alkalosis, muscle weakness, fatigue, polyuria, and polydipsia. Patients may also experience headaches, cardiac arrhythmias, and muscle cramps related to electrolyte disturbances. Adrenocortical carcinoma in general is a rare and aggressive malignancy, and cases presenting with pure aldosterone hypersecretion represent only a small fraction of all adrenocortical carcinomas. Diagnosis is challenging because the hormonal profile may initially mimic a benign aldosterone-producing adenoma. However, adrenocortical carcinomas tend to be larger at presentation (often >4 cm), may show local invasion or distant metastases, and demonstrate characteristic features on imaging and histopathology. The Weiss scoring system and Ki-67 proliferation index are commonly used to confirm malignancy. Elevated plasma aldosterone with suppressed renin activity, combined with imaging findings suggestive of a large or invasive adrenal mass, should raise suspicion for this diagnosis. Treatment is primarily surgical, with complete resection (adrenalectomy) being the cornerstone of therapy when feasible. In cases of advanced or metastatic disease, adjuvant therapy with mitotane (an adrenolytic agent) is often employed, sometimes in combination with cytotoxic chemotherapy (etoposide, doxorubicin, and cisplatin). Mineralocorticoid receptor antagonists such as spironolactone or eplerenone are used to manage hypertension and hypokalemia prior to surgery or in inoperable cases. Prognosis depends on tumor stage at diagnosis, completeness of surgical resection, and response to adjuvant therapy, but adrenocortical carcinomas generally carry a guarded prognosis, particularly in advanced stages.

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