{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T10:20:01Z","timestamp":1792059601802,"version":"4.4.0"},"reference-count":21,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,9,7]],"date-time":"2006-09-07T00:00:00Z","timestamp":1157587200000},"content-version":"vor","delay-in-days":37,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2006,8]]},"abstract":"<jats:p><jats:bold><jats:sc>Abstract:\u2002<\/jats:sc><\/jats:bold>  <jats:bold>Paragangliomas are rare tumors that arise from extra\u2010adrenal 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. Paragangliomas that hypersecrete catecholamines may cause signs and symptoms identical to those in patients with hyperfunctioning adrenal pheochromocytoma. When a catecholamine\u2010secreting tumor is suspected in a patient because of paroxysmal symptoms, biochemical documentation of catecholamine and fractionated metanephrine hypersecretion should precede any form of imaging study. Catecholamine\u2010secreting paragangliomas are found where chromaffin tissue is located (e.g., along the para\u2010aortic sympathetic chain, or within the organs of Zuckerkandl at the origin of the inferior mesenteric artery, the wall of the urinary bladder, and the sympathetic chain in the neck or mediastinum). As many as 50% of paragangliomas are hereditary and may be associated with familial paraganglioma, neurofibromatosis Type 1, von Hippel\u2013Lindau disease, the Carney triad, and, rarely, with multiple endocrine neoplasia Type 2. 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.<\/jats:bold> <\/jats:p>","DOI":"10.1196\/annals.1353.002","type":"journal-article","created":{"date-parts":[[2006,11,14]],"date-time":"2006-11-14T00:28:06Z","timestamp":1163464086000},"page":"21-29","source":"Crossref","is-referenced-by-count":178,"title":["Paragangliomas"],"prefix":"10.1111","volume":"1073","author":[{"suffix":"Jr","given":"WILLIAM F.","family":"YOUNG","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,9,7]]},"reference":[{"key":"e_1_2_8_2_2","first-page":"38","volume-title":"Histological Typing of Endocrine Tumors","author":"Lack E.E.","year":"2000"},{"key":"e_1_2_8_3_2","first-page":"268","article-title":"Extra\u2010adrenal pheochromocytoma: literature review and report of a cervical pheochromocytoma","volume":"63","author":"Fries J.G.","year":"1968","journal-title":"Surgery"},{"key":"e_1_2_8_4_2","first-page":"136","volume-title":"World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Endocrine Organs","author":"Lloyd R.V.","year":"2004"},{"key":"e_1_2_8_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(17)37119-7"},{"key":"e_1_2_8_6_2","first-page":"802","article-title":"Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979","volume":"58","author":"Beard C.M.","year":"1983","journal-title":"Mayo Clin. 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