Paragangliomas: etiology, presentation, and management

Abstract

Paragangliomas are catecholamine-secreting tumors arising from the chromaffin cells of the sympathetic ganglia, and are known as extra-adrenal pheochromocytomas. These tumors commonly present with episodic hypertension, tachycardia, headache, and diaphoresis, and can be either benign or malignant. Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging. Although the majority of paragangliomas are sporadic, a growing percentage of cases are found to be part of a familial genetic syndrome. Genetic testing should be offered to patients diagnosed with paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy. Management of paraganglioma is predicated on surgical resection, and careful perioperative management with alpha- and beta-adrenergic blockade is imperative for optimal outcomes. The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit. Long-term follow-up is essential, as paragangliomas can recur many years after initial diagnosis. Ongoing research into the genetic underpinnings of this tumor may allow for more targeted molecular therapies in the future.