Clinical Diagnosis and Management of Pheochromocytoma

Introduction to Pheochromocytoma Diagnosis

The American College of Cardiology and the American Heart Association recommend screening for pheochromocytoma using plasma free metanephrines or 24-hour urinary fractionated metanephrines as the first-line biochemical test in patients presenting with the classic triad of headache, palpitations, and sweating along with hypertension, which has 93.8% specificity and 90.9% sensitivity for the diagnosis 1, 2.

The American College of Cardiology suggests considering screening in patients with resistant hypertension, where prevalence reaches up to 4% 1, 2.
The European Society of Cardiology recommends considering screening in patients with paroxysmal or sustained hypertension with episodic symptoms including severe headaches, profuse sweating, palpitations, and pallor 1, 2, 4.
The American Heart Association advises considering screening in patients with early-onset hypertension in patients <30 years of age 1, 2.

The American College of Cardiology recommends measuring plasma free metanephrines (sensitivity 96-100%, specificity 89-98%) or 24-hour urinary fractionated metanephrines (sensitivity 86-97%, specificity 86-95%) as the initial diagnostic test 1, 2.
The European Society of Cardiology suggests that for plasma free metanephrines, ideally collect from an indwelling venous catheter after the patient has been lying supine for 30 minutes to minimize false positives 7.

The American Heart Association recommends discontinuing tricyclic antidepressants and avoiding sympathomimetics, decongestants, and certain neuropsychiatric agents before testing 1, 2, 7.

The American College of Cardiology recommends that if levels are ≥4 times the upper limit of normal, results are consistent with pheochromocytoma/paraganglioma—proceed immediately to imaging to localize the lesion 3, 5, 6.

The European Society of Cardiology recommends that once biochemically confirmed, MRI of abdomen/pelvis is preferred over CT due to risk of hypertensive crisis with IV contrast 1, 2, 3, 5.

The American Heart Association warns to never initiate beta-blockade alone before alpha-blockade in suspected pheochromocytoma, as this can precipitate severe hypertensive crisis due to unopposed alpha-adrenergic stimulation 3.

The American College of Cardiology recommends considering genetic testing in approximately 25-33% of cases with family history of pheochromocytoma/paraganglioma, young age at diagnosis, bilateral or multifocal disease, extra-adrenal location, or SDHB mutations 3, 5.

The American Heart Association recommends that if pheochromocytoma is confirmed and surgery is planned, alpha-adrenergic blockade must be started 7-14 days preoperatively 3, 5.