Natural history of cervical paragangliomas: outcomes of observation of 43 patients.

Objective: To evaluate the outcomes, growth rate, and symptoms of nonoperated cervical paragangliomas.

Design: Retrospective review of clinical and radiologic records and images.

Setting: Tertiary academic medical center.

Patients: We studied all patients presenting with cervical paragangliomas between 1993 and 2010 who were observed rather than operated on.

Main Outcome Measures: Growth of tumors and need for surgical removal.

Results: Forty-three patients with 47 tumors were identified. Mean patient age was 56 years (age range, 17-86 years). Thirty patients were asymptomatic (70%) (22 diagnosed by imaging for other reasons and 8 for workup of a neck mass), and only 5 presented with cranial nerve abnormalities (12%). No patients presented with lymphadenopathy, rapid growth, or pain. Reasons for observation were patient preference (n = 15; 35%), advanced age of patient (n = 12; 28%), and preexisting contralateral cranial nerve deficits (n = 11; 26%). Twenty-eight of the 47 tumors were suspected carotid body tumors based on imaging (60%), and 19 were suspected vagal tumors (40%). The mean greatest dimension at presentation was 2.6 cm (range, 1-7.2 cm). During a mean follow-up of 5 years (range, 1-17 years), 19 tumors remained stable in size (42%); 17 grew (38%); and 9 regressed (20%). Of the 17 tumors that grew, the mean growth was 0.2 cm/y.

Conclusions: Observation of cervical paragangliomas is an option in selected patients who do not present with worrisome symptoms. The natural history of paragangliomas in these patients is typically little to no growth over time. Regular follow-up is important to ensure minimal change and stable symptoms.