Secondary hypertension from the adrenal gland: screening tests, imaging, preoperative blockade, adrenal incidentaloma workup, and congenital adrenal hyperplasia variants.
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- What is the first-line biochemical screening test for pheochromocytoma?
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Plasma free metanephrines (or 24-hour urine fractionated metanephrines).- Why are metanephrines preferred over catecholamines for pheochromocytoma screening?
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Metanephrines are produced continuously inside the tumor, so levels are less affected by episodic secretion than catecholamines are.- What is the classic symptom triad of pheochromocytoma?
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Episodic headache, sweating, and palpitations or tachycardia.- What is the first-line imaging study to localize a suspected pheochromocytoma?
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CT or MRI of the abdomen and pelvis.- Which functional imaging study is used when a pheochromocytoma is biochemically confirmed but CT or MRI is negative, or metastatic disease is suspected?
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MIBG scintigraphy (or DOTATATE PET).- What is the first step in preoperative medical management of pheochromocytoma?
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Alpha-adrenergic blockade (for example, phenoxybenzamine or doxazosin).- Why must alpha blockade be started before beta blockade in pheochromocytoma?
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Beta blockade alone leaves alpha receptors unopposed, which can trigger a hypertensive crisis.- About how long is alpha blockade typically continued before adrenalectomy for pheochromocytoma?
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About 7 to 14 days.- What dietary advice is given during preoperative alpha blockade for pheochromocytoma, and why?
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Liberal salt and fluid intake, to expand blood volume and prevent severe post-operative hypotension.- In the classic rule of tens for pheochromocytoma, what fraction of cases are bilateral?
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About 10 percent.- In the classic rule of tens for pheochromocytoma, what fraction of cases are malignant?
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About 10 percent.- What is the term for a pheochromocytoma arising outside the adrenal gland?
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Paraganglioma.- Pheochromocytomas or paragangliomas caused by SDHB mutations carry a higher risk of what?
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Malignant, metastatic disease.- What is the definitive treatment for pheochromocytoma?
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Laparoscopic adrenalectomy, after adequate alpha blockade.- What clinical clue should prompt screening for primary aldosteronism?
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Hypertension with unexplained hypokalemia, or resistant hypertension.
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