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.