RT Book, Section A1 Myneni, Neelima A2 Atchabahian, Arthur A2 Gupta, Ruchir SR Print(0) ID 57261489 T1 Chapter 76. Pheochromocytoma T2 The Anesthesia Guide YR 2013 FD 2013 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-176049-2 LK accessanesthesiology.mhmedical.com/content.aspx?aid=57261489 RD 2024/03/28 AB Tumors of the adrenal medulla that produce, secrete, and store catecholaminesNorepinephrine is predominantly secreted along with small amounts of epinephrine, and occasionally dopaminePerioperative mortality has been reported to be as high as 45% from cardiovascular causes, and directly correlates with tumor size and degree of catecholamine secretion. With appropriate management, mortality is very lowSurgical exploration is curative in 95% of cases, with reduction in mortality to 3%Rule of 10s: 10% are malignant, 10% are extra-adrenal, 10% are bilateralOccasionally associated with syndromes: MEN IIA, MEN IIB, von Hippel-Lindau disease, or in rare cases, Von Recklinghausen’s disease, tuberous sclerosis, and Sturge-Weber syndromeTypical presentation is a young adult in 30s to 50s with sustained hypertension (or occasionally paroxysmal), tachycardia, palpitations, tremor, sweating, flushing, hyperglycemia (secondary to α-stimulated inhibition of insulin secretion)Cerebral vascular accidents and myocardial infarction are possibleIn patients with chest pain and dyspnea, catecholamine-induced cardiomyopathy should be ruled out. The cardiomyopathy may be reversible if the catecholamine stimulation is removed early before fibrosis has occurredDiagnosis: elevated plasma levels of free catecholamines and elevated urinary vanillylmandelic acid (VMA) levels along with CT findingsMIBG (methyl-iodo-benzyl-guanidine) scan may be needed to locate tumor(s)