Pheochromocytomas/paragangliomas (PPGLs) are catecholamine secreting tumors that, if missed or not properly treated, can be devastating due to potential lethal cardiovascular complications. The 2014 Endocrine Society clinical practice guideline on PPGL includes recommendations regarding optimalperi-operative care . The main goal of preoperative management is to prevent a patient from developing anesthesia- or surgery-induced catecholamine storm. This warrants the involvement of an expert team of endocrinologists, surgeons and anesthesiologists. It is recommended that all patients with a hormonally functional PPGL should undergo preoperative adrenergic blockade, primarily with alpha-adrenergic receptor blockers such as phenoxybenzamine, doxazosin, prazosin or terazosin. During 1014 days prior to surgery, doses are gradually increased until blood pressure is steadily controlled. Beta-adrenergic blocking agents such as propranolol, atenolol and metoprolol are needed when significant tachycardia or a catecholamine-induced arrhythmia occurs. A beta-blocker should not be used in the absence of an alpha-blocker because this will exacerbate epinephrine-induced vasoconstriction by blocking its vasodilator component. Calcium channel blockers or, in rare cases, the tyrosine hydroxylase inhibitor metyrosine, can be added when maximum doses of alpha- blockers are insufficient to achieve hemodynamic control. Salt and fluid loading are given to expand blood volume to prevent pre-operative orthostatic hypotension and severe post-operative hypotension. Anesthesiological management should be in experienced hands. Despite pre-operative blockade, intra-operative blood pressure may still vary, necessitating the use of short acting drugs such as phentolamine, nicardipine and sodium nitroprusside. Post-operatively the patient is monitored in the ICU for at least 24 hours to manage potential complications such as hypotension and hypoglycaemia.
 Lenders, et al., J Clin Endocrinol Metab, 2014. 99(6): pp. 191542.
18 - 21 May 2019
European Society of Endocrinology