RT Book, Section A1 Giovanni, Monica A. A1 Annes, Justin P. A2 Murray, Michael F. A2 Babyatsky, Mark W. A2 Giovanni, Monica A. A2 Alkuraya, Fowzan S. A2 Stewart, Douglas R. SR Print(0) ID 1102700751 T1 Hereditary Pheochromocytoma and Paraganglioma T2 Clinical Genomics: Practical Applications in Adult Patient Care YR 2014 FD 2014 PB McGraw-Hill Education PP New York, NY SN 9780071622448 LK accessmedicine.mhmedical.com/content.aspx?aid=1102700751 RD 2024/03/29 AB Disease summary:Paraganglia are clusters of neuroendocrine cells that comprise the sympathetic ganglia, the parasympathetic ganglia, and the adrenal medulla. A paraganglioma (PGL) is a tumor that derives from paraganglia.The term pheochromocytoma (PHEO) is applied to catecholamine-secreting paragangliomas of the adrenal gland.Extra-adrenal paragangliomas (ePGLs) may be categorized as either sympathetic (usually found in the abdomen) or parasympathetic (usually found in the head and neck; hnPGL) paragangliomas.PHEOs and ePGLs most commonly present with hypertension, headache, anxiety, and/or palpitations.hnPGLs usually present as an enlarging mass or with a mass effect such as a cranial nerve palsy (eg, Horner syndrome).PGLs have an estimated prevalence of 1 in 5000 and an estimated incidence of 1 in 30,000.