The paraganglion system comprises several groups of structures of neural crest origin, characterized by the presence of granule-storing chief cells. The classification of the extra-adrenal paraganglion system is based on the anatomic distribution, innervation, and microscopic structure into branchiomeric, intravagal, aorticosympathetic, and visceroautonomic paraganglia (6). Branchiomeric and intravagal paraganglia most commonly refer to chemoreceptors of the carotid bodies and aortic arch as well as the paraganglia of the head, neck, and superior mediastinum. Pheochromocytomas in these locations are called carotid body and glomus jugulare tumors. Tumors arising from these bodies are referred to as paragangliomas, and when functional are referred to as extra-adrenal pheochromocytomas or functional paragangliomas (7,8). Visceroautonomic paraganglia are found in the atrial septa, liver hilum, and bladder submucosa and associated with mesenteric vessels (6).

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