A 45-year-old woman comes to the office due to a 4-month history of headaches and blurred vision. She takes no medications and has no other medical conditions. After the initial laboratory work-up, a CT scan of the abdomen is performed that reveals a 2-cm left-sided adrenal mass. The patient undergoes an uncomplicated left-sided adrenalectomy. Gross examination of the adrenal gland demonstrates a well-defined yellowish tumor within the cortex. Further evaluation reveals that the tumor cells are functionally similar to cells from the outermost hormone-producing layer of the adrenal cortex. This patient would most likely have had which of the following additional symptoms?
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This patient with headaches and blurred vision (suggestive of severe hypertension) underwent resection of an adrenocortical adenoma (well-defined, yellowish tumor). Hyperfunctioning adrenocortical adenomas secrete hormones depending on the location of the cells within the adrenal cortex. In this patient, the tumor cells arise from the outermost layer (zona glomerulosa), which is responsible for producing aldosterone.
Hypersecretion of aldosterone by an adrenocortical adenoma is a common cause of primary hyperaldosteronism (Conn syndrome). Aldosterone, which is normally regulated by angiotensin II and potassium (K+) levels, stimulates reabsorption of sodium (Na+) and secretion of K+ and hydrogen (H+) in the renal collecting ducts. Excess aldosterone therefore causes increased intravascular volume and hypertension, in addition to predisposing to metabolic alkalosis and hypokalemia. Hypokalemia can cause muscle cramps, muscle weakness, and paresthesia due to hyperpolarization, which impairs membrane depolarization.
Despite increased Na+ reabsorption, extravascular volume overload (weight gain, peripheral edema) rarely occurs due to aldosterone escape. With aldosterone escape, increased intravascular volume increases pressure natriuresis and augments atrial natriuretic peptide release, thereby limiting net Na+ retention and extravascular fluid accumulation.
(Choice A) Diarrhea and flushing may be seen in carcinoid syndrome, a rare condition most often caused by a serotonin (5-HT)-secreting tumor derived from neuroendocrine cells of the gastrointestinal tract.
(Choice B) Excessive hair growth (hirsutism) can result from excess androgen production. An adrenal tumor causing androgen hypersecretion would have the functionality of the innermost layer of the adrenal cortex (zona reticularis) rather than the outermost layer as in this patient.
(Choice D) Sweating and tremulousness can be caused by excess circulating catecholamines such as from a pheochromocytoma, which can also cause headaches and blurred vision as in this patient. However, these tumors arise from the adrenal medulla rather than the adrenal cortex.
(Choice E) Patients with Cushing syndrome often develop weight gain (due to central fat accumulation) and have easy bruising (due to thinning of subcutaneous connective tissue). Cortisol-secreting adrenal tumors would be functionally similar to the middle layer of the adrenal cortex (zona fasciculata).
Educational objective:
Hypersecreting adrenocortical tumors can have functionality resembling the outer (aldosterone), middle (cortisol), or inner (androgens) layers of the adrenal cortex. Primary hyperaldosteronism results in hypertension in addition to hypokalemia, which can cause muscle cramps, muscle weakness, and paresthesia.