Management of paragangliomas in otolaryngology practice: review of a 7-year experience

Abstract

Background and aims: Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas) and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors.

Materials and methods: Twenty-six patients with 27 paragangliomas were treated in our institution during a period of 7 years (2000-2007). There were 15 women (57.6%) and 11 men (42.4%) with a mean age of 33.5 years. A painless lateral neck mass was the main finding in 16 patients (61.5%). There was no evidence of a functional tumor. Carotid angiography was performed on all of our patients (100%) to define the vascular anatomy of the lesion. Twenty-two paragangliomas (of the 25 operated paragangliomas; 88%) underwent selective embolization of the major feeding arteries. We performed surgery on 24 (92.3%) patients. Two patients were treated with radiotherapy.

Results: Most lesions were paragangliomas of the carotid bifurcation (n = 14 [51.8%]), whereas 6 patients were diagnosed with jugular (22.2%), 1 with a vagal (3.7%), 1 with a tympanic paraganglioma (3.7%), 2 with jugulotympanic paraganglioma (7.4%), and 1 with laryngeal paraganglioma (3.7%). In 1 patient (3.8%), bilateral paragangliomas in the carotid bifurcation were detected. There was an evidence of malignancy in all cases (3.8%). Preoperative embolization has proven successful in reducing tumor vascularity in approximately 22 (of 25 who accepted surgery; 88%) paraganglioma patients. The common preoperative complication was vascular injury, which occurred in 6 (23%) of 26 patients; the main postoperative complication was transient cranial nerve deficit in 4 (15.3%) of 26 patients; and a permanent Horner syndrome was documented in 2 patients (7.6%). Cerebrospinal fluid leak occurred in 1 patient (3.7%). Postoperatively, stroke was occurred in 1 patient (3.7%). Two patients with jugular paraganglioma were treated with irradiation because of skull base extension with significant symptomatic relief.

Conclusions: The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Combined therapeutic approach with preoperative selective embolization followed by surgical resection is the safe and the effective method for complete excision of the tumors with a reduced morbidity rate.