Surgical and Pharmacological Management of Functioning Pheochromocytoma and Paraganglioma

Pheochromocytoma and paraganglioma are rare vascular neuroendocrine tumors. These tumors can synthesize and store excessive amounts of norepinephrine and epinephrine, which, when released, can produce life-threatening cardiovascular complications. Adequate evaluation, including genetic data, is crucial before surgery. The goal of preoperative management includes evaluation of cardiovascular sequelae from high levels of circulating catecholamines. Preoperative medical therapy is aimed at controlling volume expansion, hypertension, and tachycardia to avoid intraoperative hemodynamic instability. Initiation of therapy with antihypertensive agents is recommended even for normotensive patients to prevent unpredictable intraoperative hemodynamic instability. Surgical approach in these patients is often complex. An experienced surgical team is required, and several factors must be taken into account to select the best approach. Remarkable advances have been made in the surgical approach to adrenalectomy for pheochromocytomas, with a majority being performed laparoscopically and only selected cases undergo open surgery. Intraoperative management from the anesthesia team is also important to provide anesthetics that maintain hemodynamic stability. Postoperative complications may include hypotension, hypertension, and hypoglycemia and must be managed in intensive care units. This chapter describes the perioperative management of functioning abdominal pheochromocytoma/paraganglioma discussing preoperative management, surgical techniques, intraoperative pharmacological treatments, and postoperative follow-up.