Secondary Hypertension: Pheochromocytoma and Paraganglioma

Pheochromocytomas and paragangliomas are rare but important causes of secondary hypertension. Unrecognized tumors are associated with high cardiovascular morbidity and mortality. Surgery is the mainstay of treatment, and patients require appropriate perioperative alpha-blockade. Up to 40% of patients have germline pathogenic variants in known susceptibility genes. Knowing if a germline pathogenic variant is present has implications for treatment, screening, and surveillance of patients and their family members; consequently, all patients with pheochromocytomas and paragangliomas should be referred for clinical genetic testing.