Pheochromocytoma and Paraganglioma

Pheochromocytomas and paragangliomas (PPGLs) are tumors that arise from chromaffin cells and typically produce excess catecholamines. Approximately 40% of patients have a genetic mutation and 10–15% of these tumors are malignant. All patients with PPGLs should be considered for genetic testing. Patients often present with episodic symptoms such as headache, diaphoresis, and palpitations. Fractionated metanephrines are used for diagnosis. After a minimum of 7–10 days of medical treatment with alpha-adrenergic blockade, patients can safely undergo surgical resection. Since malignancy cannot be determined on final pathology, patients should be followed with yearly metanephrines to screen for recurrence or metastasis.