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Sympathetic Paragangliomas

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Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines (1). They are closely related to pheochromocytomas, which are sometimes referred to as intra-adrenal paragangliomas (2). The majority appear to be sporadic, but approximately one-third to one-half are associated with an inherited syndrome (3, 4). The four genetic syndromes that are associated are: multiple endocrine neoplasia 2A and 2B, neurofibromatosis type 1, von Hippel Lindau, and the Carney-Stratakis dyad. The male-to-female ratio is approximately equal among patients with hereditary paraganglioma, …show more content…

The majority of sympathetic paragangliomas arise outside of the skull base and neck anywhere along the sympathetic chain (8). About 75 percent arise in the abdomen, most often at the junction of the inferior vena cava and left renal vein and about 10 percent arise in the thorax (9, 10). They excrete excess amounts of catecholamine (86 percent in one series) (7), usually almost always …show more content…

Patients can become hypertensive during induction, positioning, or tumor resection (12). Chronic catecholamine excess causes volume contraction and patients can become severely hypotensive, as in this case, if adequate volume resuscitation is not performed (12). It is prudent to evaluate for adverse events following hypertensive and hypotensive episodes. Serial neurological evaluations, CT brain, electrocardiograms, or serial cardiac enzymes may be warranted. Complications of surgery are primarily due to severe preoperative hypertension, high secretion tumors, or repeat intervention for recurrence (13). In one study, adverse perioperative events occurred in 32 percent of cases (14). The most common adverse event was sustained hypertension in 25 percent of the patients. There were no perioperative deaths, myocardial infarctions, or cerebrovascular events. Despite premedication of most patients with phenoxybenzamine and a beta-blocker, varying degrees of intraoperative hemodynamic lability occurred

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