What is the management of pheochromocytoma?

From the Guidelines

The management of pheochromocytoma involves surgical removal of the tumor after appropriate preoperative preparation with alpha-adrenergic blockade, such as doxazosin or terazosin, followed by beta-blockade if necessary, as recommended by the 2024 ESC guidelines for the management of elevated blood pressure and hypertension 1.

Preoperative Preparation

Before surgery, patients require alpha-adrenergic blockade to control hypertension and prevent catecholamine-induced complications. The 2024 ESC guidelines suggest using an intravenous alpha-1-blocker, such as phentolamine, doxazosin, or terazosin, or labetalol, which has the advantage of allowing titration of the infusion based on the blood pressure response and avoids tachycardia via beta-blockade 1.

• Alpha-blockers like doxazosin or terazosin should be administered for 1-2 weeks to control blood pressure and adrenergic crises.
• Beta-blockers should be added only after adequate alpha-blockade to prevent reflex tachycardia.

Surgical Excision

• Identifying a single tumor mandates surgical excision after adequate pharmacological preparation, as secreting pheochromocytomas can cause fatal events with no warning 1.
• The definitive treatment is laparoscopic adrenalectomy, which should be performed by experienced surgeons.

Postoperative Care

• During surgery, patients require close hemodynamic monitoring as tumor manipulation can cause catecholamine surges.
• Postoperatively, blood pressure and glucose levels should be monitored as hypotension and hypoglycemia may occur after tumor removal.
• Adequate fluid administration should be carefully managed to prevent profound hypotension, particularly right after tumor excision, as patients with pheochromocytoma have peripheral hypovolaemia that exposes them to this risk 1.

Long-term Follow-up

• Long-term follow-up is necessary as approximately 10% of pheochromocytomas recur, and some patients may have genetic syndromes requiring surveillance for other tumors.
• Genetic testing should be considered for all patients with pheochromocytoma to identify hereditary forms of the disease.

From the FDA Drug Label

Metyrosine capsules are indicated in the treatment of patients with pheochromocytoma for: 1. Preoperative preparation of patients for surgery. 2. Management of patients when surgery is contraindicated. 3. Chronic treatment of patients with malignant pheochromocytoma. Phenoxybenzamine hydrochloride capsules are indicated in the treatment of pheochromocytoma, to control episodes of hypertension and sweating. If tachycardia is excessive, it may be necessary to use a beta-blocking agent concomitantly.

The management of pheochromocytoma includes:

• Preoperative preparation for surgery using metyrosine [ 2 ]
• Control of hypertension and sweating using phenoxybenzamine [ 3 ]
• Chronic treatment of malignant pheochromocytoma using metyrosine [ 2 ]
• Possible use of a beta-blocking agent in cases of excessive tachycardia [ 3 ]

From the Research

Management of Pheochromocytoma

The management of pheochromocytoma involves several key steps, including:

• Preoperative localization and pharmacologic treatment with alpha-adrenergic blockers (and occasionally with beta-adrenergic blockers and/or alpha-methylparatyrosine) 4
• Meticulous anesthesia and intraoperative cardiovascular monitoring 4
• Attention to the surgical principles of wide exposure, careful dissection and complete exploration, early interruption of tumor vasculature, and delivery of the tumor with the capsule intact 4

Pharmacologic Treatment

Pharmacologic treatment of pheochromocytoma typically involves the use of:

• Alpha-adrenergic blocking agents, such as phenoxybenzamine or doxazosin, to control blood pressure 5, 6, 7
• Beta-adrenergic blockers, such as propranolol, to control tachycardia and arrhythmias, but only after adequate alpha-adrenergic blockade 5, 7
• Alpha-methylparatyrosine, which decreases catecholamine synthesis, in cases where surgery is contraindicated or in malignant pheochromocytoma 5, 4

Perioperative Management

Perioperative management of pheochromocytoma requires careful control of blood pressure and heart rate, using:

• Selective α1-adrenergic blocking agents, such as doxazosin, prazosin, or terazosin, followed by β-adrenergic blockade 7
• Vasodilators, such as calcium channel blockers, to control blood pressure if necessary 7

Treatment of Hypertensive Crisis

Treatment of hypertensive crisis due to pheochromocytoma requires prompt control of blood pressure, using:

• Sodium nitroprusside or other vasodilators to rapidly lower blood pressure 5
• Beta-adrenergic blockers, such as propranolol, to control tachycardia and arrhythmias 5, 7