Pheochromocytoma

Pheochromocytoma is a rare cause of endocrine hypertension with potentially fatal consequences if left untreated. A high index of suspicion is required of clinicians due to the nonspecific nature of symptoms, the risk of significant morbidity and mortality if the diagnosis is missed, and the high frequency of associated familial disease. Symptomatic patients typically present with complaints consistent with sustained or intermittent catecholamine excess, although some pheochromocytomas may be clinically silent, particularly those diagnosed in the evaluation of adrenal incidentalomas. Plasma free metanephrines or 24-hour urine fractionated metanephrines are the initial screening tests of choice and, only if elevated, should be followed by imaging to localize the tumor. Treatment for a confirmed pheochromocytoma is urgent surgical resection; however blood pressure must be controlled preoperatively with α-blockers to prevent a hypertensive crisis. Following treatment, all patients with pheochromocytoma should undergo a clinical assessment for the associated familial syndromes and susceptibilities with subsequent genetic testing.