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Treatment of pheochromocytoma in adults

Authors
William F Young, Jr, MD, MSc
Electron Kebebew, MD, FACS
Section Editors
André Lacroix, MD
Sally E Carty, MD, FACS
Deputy Editors
Kathryn A Martin, MD
Wenliang Chen, MD, PhD

INTRODUCTION

Pheochromocytoma is a rare neuroendocrine tumor, occurring in less than 0.2 percent of patients with hypertension [1,2]. In at least 25 percent of patients, the tumor is discovered incidentally during computed tomography (CT) or magnetic resonance imaging (MRI) of the abdomen for unrelated symptoms [3-6]. Studies have found that 19 of 33 patients (58 percent) and 40 of 57 patients (70 percent) with adrenal pheochromocytoma had their tumors discovered incidentally on imaging [5,6]. In other patients, the tumor is found only at autopsy [7]. Among patients suspected to have a pheochromocytoma, the diagnosis is rarely confirmed. In one series, as an example, the diagnosis was established in only 1 of 300 patients evaluated for pheochromocytoma [8].

The treatment of pheochromocytoma will be reviewed here (algorithm 1). The clinical manifestations, diagnosis, and genetics of pheochromocytoma and management of metastatic pheochromocytoma are discussed separately. (See "Clinical presentation and diagnosis of pheochromocytoma" and "Pheochromocytoma in genetic disorders" and "Paraganglioma and pheochromocytoma: Management of malignant disease".)

MEDICAL PREPARATION FOR SURGERY

Once a pheochromocytoma is diagnosed, all patients should undergo a resection of the pheochromocytoma following appropriate medical preparation. Agents known to provoke a pheochromocytoma paroxysm (eg, glucagon, histamine, metoclopramide) should be avoided. Our approach is largely consistent with the Endocrine Society’s 2014 Clinical Practice Guidelines [9].

Resecting a pheochromocytoma is a high-risk surgical procedure and an experienced surgeon/anesthesiologist team is required. Cardiovascular and hemodynamic variables must be monitored closely. Continuous measurement of intra-arterial pressure and heart rhythm is required. In the setting of congestive heart failure or decreased cardiac reserve, monitoring of pulmonary capillary wedge pressure is indicated.

Preoperative medical therapy is aimed at:

                      

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Literature review current through: Nov 2016. | This topic last updated: Tue Oct 27 00:00:00 GMT+00:00 2015.
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