Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas.

Background: Current experience with carotid body tumors suggesting a high prevalence of associated cervical paragangliomas prompted this review.

Methods: An 8-year retrospective study of patients with carotid body tumors was undertaken, detailing presentation, diagnosis, and treatment.

Results: Eleven patients harboring 17 carotid body tumors were discovered. All patients had a neck mass. Seven patients (64%) had bilateral carotid body tumors. Six (55%) reported a positive family history-4 were first-generation relatives, 5 had bilateral tumors, and 3 had other head and neck paragangliomas. Angiography documented 4 associated vagal and 2 glomus jugulare paragangliomas in addition to the carotid body tumors. Precise surgical care limited blood loss to an average of 590 cc. The carotid artery was repaired during 5 resections (29%). Cranial nerve injury occurred in 3 cases, all following vagal body or glomus jugulare resection. Every patient is currently alive, stroke free, and functioning without major disability.

Conclusions: Patients with carotid body tumors have a propensity for multiple head and neck paragangliomas. Angiography is diagnostic. The need for associated paraganglioma resection dramatically increases the risk of cranial nerve injury.