Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD


Cancer Control. 2016;23(3):228-241. 

In This Article


Vascular Injury

The incidence rate for stroke following surgery for paragangliomas has been reported to be as high as 20% and as low as 2% or less.[1–3] With the evolution of preoperative planning, surgical techniques, and diagnostic evaluations, the need to sacrifice the internal carotid and the risk of injury are minimal. The risk of injury to the carotid artery or need for sacrifice following treatment of carotid body tumors is size specific: tumors larger than 5 cm are likely to require carotid reconstruction.

Unlike jugulotympanic paragangliomas and carotid body tumors, vagal paragangliomas are not closely associated with the carotid artery, although the internal carotid artery may be involved in its petrous portion in advanced disease. Rarely, injury may occur, even with adequate surgical exposure and microsurgical technique. If the patient is at high risk for vessel injury within the petrous carotid portion and balloon occlusion testing has been safely and satisfactorily performed, then the surgeon may consider permanent preoperative occlusion of the carotid distal to the tumor.[1–3]

Baroreflex Failure

Following the resection of bilateral carotid body tumors, the baroreceptor reflex is lost and bilateral denervation of the carotid sinus is unavoidable, resulting in postoperative labile refractory hypertension, tachycardia, diaphoresis, and headache.[30] The long-term treatment of choice is clonidine.[31] Algorithms have been created for the management of bilateral carotid body tumors to avoid long-term postoperative hypertensive issues.[1–3]

Cranial Nerve Injury

The surgical risk to the lower cranial nerves for the treatment of paragangliomas is site specific and directly related to tumor size. Vagal, jugulotympanic, and carotid body paragangliomas represent, in decreasing order, risk of injury to the cranial nerves. The size of the tumor is especially important in vagal and jugulotympanic paragangliomas. Tumors presenting with extensive skull-base involvement are likely to have extensive lower cranial nerve involvement and often preoperatively present with multiple cranial nerve deficits. Facial nerve involvement in conjunction with preoperative paralysis is a sign of such extensive involvement.[16,30,32–34]

Following surgery, deficits in the cranial nerve may involve impaired aspiration, deglutition, tongue motion, and phonation. Patients with more than 1 cranial nerve deficit do not respond well to treatment because these deficits have additive effects. Those who are older may experience difficulty in recovery because vocal cord medialization may be necessary if a risk of aspiration is present. In such patients, tracheostomy or gastrostomy may be necessary — this is especially true for patients with injuries to the high vagus nerve.

Injury to the accessory nerve results in functional loss of the sternocleidomastoid and trapezius muscles. Patients with such injury should be referred to physical therapy to help avoid shoulder pain secondary to shoulder "drop" and limited range of motion. If the nerve is injured below the jugular foramen, then primary repair or nerve grafting may be performed.[17]

Hypoglossal nerve injury results in paralysis of the ipsilateral tongue. Long-term hypoglossal nerve paralysis results in hemiatrophy of the tongue within a few months. If this occurs in combination with other lower cranial nerve injuries, then swallowing therapy will be required to prevent aspiration. Swallowing therapy is usually focused on educating the patient to direct the bolus to the functioning side.[17] More significant, persistent swallowing and aspiration issues may require feeding via tracheostomy and gastrostomy tubes.