Vagal paraganglioma: a review of 46 patients treated during a 20-year period.
BACKGROUND
Vagal paragangliomas (VPs) arise from paraganglia associated with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information regarding these tumors has arisen from case reports and small clinical series.


OBJECTIVE
To detail the clinicopathologic features of 46 patients with VP with an emphasis on the role of a multidisciplinary skull base team in both the successful extirpation and rehabilitation.


METHODS
Retrospective review of 46 patients with VP managed by a single skull base team.


METHODS
An academic tertiary medical center.


RESULTS
Forty-six patients were treated over a 20-year period (1978-1998). Ten (22%) demonstrated intracranial extension. There was a history of familial paragangliomas in 9 (20%) of the patients. The incidence of multicentric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of this group of 46 patients consisted of surgery (n = 40), radiation therapy (n = 4), and observation (n = 2). The operative approach consisted of a transcervical excision often combined with a transtemporal or lateral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabilitation was a vital component in the management of these tumors.


CONCLUSIONS
The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include surgical resection, radiation therapy, and, in selected cases, observation. 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 deficits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.
Department of Otolaryngology--Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, Tenn 37232-2559, USA. james.netterville@mcmail.vanderbilt.edu
J L
JL
Netterville
C G
CG
Jackson
F R
FR
Miller
J R
JR
Wanamaker
M E
ME
Glasscock
D000293:Adolescent; D000328:Adult; D000368:Aged; D003390:Cranial Nerve Neoplasms / Q000175:diagnosis* / Q000534:rehabilitation / Q000601:surgery; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000534:rehabilitation / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000175:diagnosis* / Q000534:rehabilitation / Q000601:surgery; D011182:Postoperative Care; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies; D014630:Vagus Nerve / Q000601:surgery