VAGAL PARAGANGLIOMA - A REVIEW OF 46 PATIENTS TREATED DURING A 20-YEAR PERIOD

Background: Vagal paragangliomas (VPs) arise from paraganglia associat ed with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information reg arding these tumors has arisen from case reports and small clinical se ries. Objective: To detail the clinicopathologic features of 46 patien ts with VP with an emphasis on the role of a multidisciplinary skull b ase team in both the successful extirpation and rehabilitation. Design : Retrospective review of 46 patients with VP managed by a single skul l base team. Setting: An academic tertiary medical center. Results: Fo rty-six patients were treated over a 20-year period (1978-1998). Ten ( 22%) demonstrated intracranial extension. There was a history of famil ial paragangliomas in 9 (20%) of the patients. The incidence of multic entric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of thi s group of 46 patients consisted of surgery (n = 40), radiation therap y (n = 4), and observation (n = 2). The operative approach consisted o f a transcervical excision often combined with a transtemporal or late ral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabili tation was a vital component in the management of these tumors. Conclu sions: The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include su rgical resection, radiation therapy, and, in selected cases, observati on. Surgical extirpation requires a multidisciplinary skull base team to achieve complete tumor resection. Radiation therapy is reserved for elderly patients and patients at risk for bilateral cranial nerve def icits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.