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KMID : 0361020000430040442
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Korean Journal of Otolaryngology - Head and Neck Surgery
2000 Volume.43 No. 4 p.442 ~ p.446
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Multiple Paragangliomas ; Three Cases
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Seo Min-Cheol
Lee Kwang-Sun
Kim Chang-Jin
Kim Sang-Yoon
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Abstract
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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.
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KEYWORD
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Multiple paraganglioma, Bilateral carotid body tumor, Family history
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