Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD


Cancer Control. 2016;23(3):228-241. 

In This Article

Anatomy and Physiology

Diffusely distributed throughout the upper part of the body, paraganglia facilitate the chemoreceptive reflexes of the cardiovascular system. They contain chief cells with the capacity to secrete neuropeptides (eg, norepinephrine), which, in turn, influence vascular reflexes.[1–3]

The carotid body, which is a type of paraganglion, can sense changes in pH, arterial oxygen pressure, and level of carbon dioxide.[1–3] Located within the carotid bifurcation, the carotid body is a discrete, oval structure that directly receives its blood supply from the carotid bifurcation via the glomic arteries (Fig 1).[3] The afferent reflex is mediated by a glossopharyngeal nerve branch called the nerve of Hering. Expanding tumors that originate from here can impair sympathetic nerve chain function as well as cranial nerves X and XII.[1–3]

Figure 1.

Schematic representation of common sites of head and neck paragangliomas and their relationship to the lower cranial nerves and major vessels.
From Persky MS, Hu KS. Paragangliomas of the head and neck. In: Harrison LB, Sessions RB, Hong WK, eds. Head and Neck Cancer: A Multidisciplinary Approach. 3rd ed. Lippincott Williams and Wilkins, Philadelphia, PA; 2009. Reprinted with permission by Wolters Kluwer.

Distributed within the temporal bone in close association with the Jacobsen nerve, which is the tympanic branch of the glossopharyngeal nerve (see Fig 1), are the jugulotympanic paraganglia.[3] Typically, temporal bone paraganglia are located in the jugular fossa, and symptoms may involve early functional impairment of cranial nerves IX, X, XI within the jugular foramen, and XII as it exits the hypoglossal canal.[1–3]

Vagal paraganglia are distinctly separate from jugulotympanic paraganglia because they do not form discrete bodies; in addition, they may be interspersed within the vagal nerve fibers in the pars nervosa of the jugular foramen (which transmits lower cranial nerves IX, X, and XI) or located within the vagus nerve beneath the perineurium.[1–3] The superior vagal ganglion is visible at the level of the jugular foramen. The origin of most vagal paragangliomas is the nodose vagal ganglion, which is located approximately 1 to 2 cm below the jugular foramen (see Fig 1).[3] Both the superior and nodose vagal ganglia are proximal to the pars venosa of the jugular foramen, cranial nerves IX to XII, and the ascending portion of the petrous internal carotid artery.[15] Therefore, vagal paragangliomas have distinct therapeutic sequela based on their close anatomical association with the superior portion of the vagal nerve and other adjacent neurovascular structures.[1–3]

Extra-adrenal paraganglia lack methyltransferase, a requirement for converting norepinephrine to epinephrine; the metabolic breakdown of catecholamines to metanephrine (from epinephrine) and normetanephrine (from norepinephrine) as well as vanillylmandelic acid can be detected in urine. Thus, appropriate urine and serum analysis can be used to detect actively secreting paragangliomas.[1–3]