Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD


Cancer Control. 2016;23(3):228-241. 

In This Article


History and Physical Examination

The health care professional should obtain a thorough history and perform a physical examination for any patient presenting with a paraganglioma, evaluating for cranial nerve dysfunction, possible signs and symptoms related to catecholamine secretion as well as evidence of malignant transformation.[1–3] Typically, carotid body tumors present as an enlarging asymptomatic mass at the level of the carotid bifurcation. Dysfunction of the cranial nerve may be indicative of the presence of large tumors extending into the jugular foramen.[1–3] Patients with jugulotympanic tumors may present with pulsatile tinnitus or conductive hearing loss. Otoscopic examination may reveal the Brown sign involving a red-blue lesion in the middle ear that blanches with positive pressure.[1–3] For patients with more advanced disease, lower cranial nerve deficits may become apparent. Frequently (< 50% of cases), vagal paragangliomas present with multiple cranial neuropathies involving the hypoglossal, vagus, and spinal accessory nerves.[3,13,16,17]

Attention should be given to a possible familial history of paragangliomas, von Hippel–Lindau syndrome, and type 2 multiple endocrine neoplasia.[1–3] Sweating, hypertension, tachycardia, and nervousness may be symptoms of secreting tumors. Laboratory studies, including 24-hour urinalysis and serum catecholamine screening (norepinephrine, epinephrine, and metanephrine), should be ordered for all patients with suspected paraganglioma.[1–3]

Diagnostic Imaging

In general, fine needle biopsy is not indicated because radiographic studies are pathognomonic.[1–3,16,18] The health care professional can obtain computed tomography (CT) with contrast for delineating paragangliomas because the contrast enhances these highly vascular tumors.[1–3] In addition, CT is frequently diagnostic given the characteristic patterns tumors displace to the internal and external carotid arteries.[3] CT can be utilized to define the erosion and any possible skullbase involvement, including in the temporal bone. A well-circumscribed mass occupying the carotid bifurcation that has splayed the external and internal carotid arteries is characteristic of carotid body tumors, which posterolaterally displace the internal carotid artery. Typically, when vagal paragangliomas are present, the internal carotid artery is anteriorly/medially displaced, occupying the superior parapharyngeal space (with or without skull-base involvement).[1–3] In the early disease stage, jugulotympanic paragangliomas can be distinguished — in particular when a paraganglioma is located in the tympanic cavity alone — and bone destruction patterns typical for this type of paraganglioma are observed.[1–3]

MRI with gadolinium contrast is a complementary imaging modality that the health care professional can also employ for evaluating head and neck paragangliomas.[1–3] Gadolinium should be used, if available, because paragangliomas show intense signal enhancement in this medium.[19] Similar to CT, use of contrast for MRI aids the health care professional in the delineation of the tumor, and it also helps to survey and detect synchronous paragangliomas of the head and neck as well as confirm the diagnosis. On T2-weighted MRI, the characteristic "salt and pepper" appearance observed is essentially pathognomonic and is related to the high-flow vascular voids within the vascular tumor.

Findings on magnetic resonance angiography that demonstrate the patency of the circle of Willis provide the surgeon with critical information about intracranial circulation. Angiography can be used to detail the features of tumor flow dynamics (the venous drainage and blood supply of the tumor venous drainage) as well as the vascular anatomy of the intracranial space and head and neck (Fig 2).[3] If carotid artery sacrifice is anticipated, then cerebral angiography with ipsilateral internal carotid balloon occlusion can be obtained to define the intracranial cerebral circulation. However, because techniques in high-resolution MRI and CT have evolved, the role of magnetic resonance angiography is limited in diagnosis, although it does remain important for use in preoperative planning for tumor resection. Obtaining superselective angiography and performing embolization of the arterial supply can decrease the risk for intraoperative blood loss.

Figure 2.

Carotid body tumor splaying the carotid bifurcation.
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.

Radionuclide imaging can be used to target the biochemical pathways of catecholamine synthesis. Such imaging techniques include fluorodeoxyglucose with or without metaiodobenzylguanidine, dihydroxyphenylalanine, fluorodopamine, fluorodopa, or indium octreotide for use with PET.[20,21]