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Paragangliomas: Treatment of locoregional disease

Sally E Carty, MD, FACS
William F Young, Jr, MD, MSc
Kevin Oh, MD
Section Editors
Patrick Y Wen, MD
Jay S Loeffler, MD
André Lacroix, MD
Deputy Editor
Sadhna R Vora, MD


Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs that consist mainly of neuroendocrine (chromaffin) cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines (figure 1). (See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Catecholamine hypersecretion'.)

Paragangliomas are closely related to pheochromocytomas, which are sometimes referred to as intra-adrenal paragangliomas, and they are indistinguishable from pheochromocytomas at the cellular level. Catecholamine-secreting paragangliomas often present clinically like pheochromocytomas with hypertension, episodic headache, sweating, and tachycardia. However, the distinction between pheochromocytoma and paraganglioma is an important one because of implications for associated neoplasms, risk for malignancy, and genetic testing. (See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Histology and malignant potential' and "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Indications for genetic testing'.)

Paragangliomas can derive from either parasympathetic or sympathetic paraganglia. They differ in their anatomic distribution, frequency of an underlying genetic syndrome, and clinical features (see "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Definition and anatomic origin'):

The majority of parasympathetic ganglia-derived paragangliomas are located in the neck and skull base along the branches of the glossopharyngeal and vagus nerves (figure 2). They arise most commonly from the carotid body, less commonly from jugulotympanic and vagal paraganglia, and rarely from the laryngeal paraganglia.

A minority of skull base paragangliomas (approximately 5 percent) are symptomatic from hypersecretion [1,2]. About one-half are associated with a disease-causing germline mutation. (See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Hereditary syndromes' and "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology", section on 'Catecholamine hypersecretion'.)

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Literature review current through: Nov 2017. | This topic last updated: May 12, 2016.
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