Annals of the New York Academy of Sciences
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Paragangliomas are rare tumors that arise from extra-adrenal paraganglia. The effective diagnosis and management of the paraganglioma patient involves the close collaboration of endocrinologists, endocrine surgeons, anesthesiologists, geneticists, laboratory specialists, radiologists, oncologists, and pathologists. Paragangliomas are diagnosed in the following clinical settings: signs and symptoms related to catecholamine hypersecretion, mass effect symptoms (e.g., with head and neck paragangliomas), incidental finding on imaging, or family screening for hereditary paraganglioma. ⋯ Genetic testing should be considered in all patients with paraganglioma. The treatment of choice for paraganglioma is surgical resection; most tumors are benign and can be excised totally. Following surgical cure, annual biochemical testing assesses for metastatic disease, tumor recurrence or delayed appearance of multiple primary tumors.
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Ann. N. Y. Acad. Sci. · Aug 2006
ReviewAn overview of pheochromocytoma: history, current concepts, vagaries, and diagnostic challenges.
Tragically as many as 50% of pheochromocytomas are discovered at autopsy, mainly because the diagnosis of this neuroendocrine tumor was not considered. Missing the diagnosis almost invariably results in devastating cardiovascular complications or death. ⋯ Very rarely, familial pheochromocytomas may cause no hypertension, symptoms, or signs. But biochemical testing can always establish the presence or absence of a pheochromocytoma, and localization with magnetic resonance imaging, computed tomography, or 131I or 123I-MIBG is almost always possible.