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- W2145280480 abstract "Objective: To report a case of a woman with poorly controlled hypertension who was found to have multiple paragangliomas and mutation in the B subunit of succinate dehydrogenase (mitochondrial complex II). Methods: The pathogenesis of familial paragan gliomas and case management are discussed, and the rele vant literature is reviewed. Results: In a 68-year-old woman with a long-term history of hypertension, bilateral carotid body paragan gliomas had been resected at 24 and 57 years of age. Current laboratory studies showed an elevated plasma normetanephrine level. Computed tomography and mag netic resonance imaging revealed 3 paragangliomas—1 in the middle mediastinum and 2 intra-abdominally. Surgical excision involved a 2-stage procedure with the patient under the same general anesthesia: ( 1) a median sternoto my without cardiopulmonary bypass and ( 2) a midline celiotomy for removal of the retroperitoneal paragan gliomas. All 3 tumors were focally adherent to adjacent structures (the left atrium, the uncinate process of the pan creas, and the left renal vessels). All tumors were com pletely excised without complication, and frozen section analysis confirmed that they were paragangliomas. Genetic testing disclosed a germline mutation inSDHB . Conclusion: Familial paraganglioma is a rare autoso mal dominant, tumorigenic disorder affecting sympathetic and parasympathetic paraganglia. (Endocr Pract. 2008; 14:603-606)" @default.
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- W2145280480 date "2008-07-01" @default.
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- W2145280480 title "Familial Paragangliomas: Case Report and Literature Review" @default.
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- W2145280480 doi "https://doi.org/10.4158/ep.14.5.603" @default.
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