Multiple Paragangliomas: Three Cases / 대한이비인후과학회지

ABSTRACT

Paragangliomas are neoplasms that arise from extra-adrenal paraganglia, microscopic islands of cells derived from the neural crest. In the head and neck region, paraganglionic tissue is distributed in the superior and inferior paraganglia, the carotid body, the vagal body, and the jugulotympanic region. Approximately 10% of patients with a paraganglioma have a family history of such tumors. Multiple lesions can be found in 26% of patients with family history. Patients with multiple paragangliomas have a higher risk of having functional pheochromocytoma and should undergo a pretreatment screening for vasopressor substances. Similarly, patients with a family history should undergo four-vessel arteriography to rule out any multiple and clinically unrecognized lesions. The authors have experienced three cases of multiple paragangliomas, which were managed surgically. Two cases were bilateral carotid body tumors and two cases were accompanied by glomus jugulare. Of these, one case had family history. We report three cases of multiple paragangliomas with a review of literatures.