What is the urgency of treatment for pheochromocytoma?

From the Guidelines

Treatment for pheochromocytoma is urgent and should not be delayed once diagnosed, as these tumors can cause fatal events with no warning. The management of pheochromocytoma involves surgical excision after adequate pharmacological preparation, as secreting pheochromocytomas can cause life-threatening complications including hypertensive crisis, stroke, heart attack, or heart failure 1. According to the 2024 ESC guidelines for the management of elevated blood pressure and hypertension, identifying a single tumor mandates surgical excision after adequate pharmacological preparation 1.

Key considerations in the management of pheochromocytoma include:

• Administering alpha-blockers, such as doxazosin or terazosin, to control blood pressure and adrenergic crises 1
• Adding beta-blockers after alpha-blockade is established to prevent worsening hypertension 1
• Ensuring adequate fluid administration to prevent profound hypotension, particularly right after tumor excision, as patients with pheochromocytoma have peripheral hypovolaemia 1
• Using intravenous alpha-1-blockers, such as phentolamine, doxazosin, or terazosin, or labetalol, to treat adrenergic crises and hypertensive emergencies 1

The urgency of treatment stems from the unpredictable nature of catecholamine release from these tumors, which can cause sudden, severe, and potentially fatal blood pressure spikes 1. Even with seemingly stable patients, the risk of catastrophic events makes prompt, systematic treatment essential. Surgical excision of the tumor is the definitive treatment, and delaying treatment can result in increased morbidity and mortality.

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. In patients with pheochromocytoma, who produce excessive amounts of norepinephrine and epinephrine, administration of one gram to four grams of metyrosine per day has reduced catecholamine biosynthesis from about 35% to 80% as measured by the total excretion of catecholamines and their metabolites

The urgency of treatment for pheochromocytoma is implied to be high due to the need for:

• Preoperative preparation to prevent potential complications during surgery
• Management when surgery is contraindicated, suggesting that treatment is necessary to control symptoms
• Chronic treatment for malignant cases, indicating ongoing management is required 2, 2 However, the exact level of urgency is not explicitly stated in the provided drug labels.

From the Research

Urgency of Treatment for Pheochromocytoma

The urgency of treatment for pheochromocytoma is a critical aspect of managing this condition. According to the available evidence:

• Pheochromocytoma crisis is not considered a surgical emergency 3. Instead, initial stabilization of the acute crisis followed by sufficient α-blockade before surgery is recommended.
• Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality 3.
• Preoperative treatment with alpha-blockers, such as phenoxybenzamine or doxazosin, is essential to prevent hemodynamic instability during surgery 4, 5.
• The choice of alpha-blocker may affect the outcome, with phenoxybenzamine providing better control of systolic arterial pressure but also causing more pronounced postoperative hypotension 5.
• Selective vs non-selective alpha-blockade prior to adrenalectomy for pheochromocytoma has been compared in a systematic review and meta-analysis, which found that non-selective alpha-blockade was more effective in preventing intraoperative blood pressure fluctuations 6.

Key Considerations

Some key considerations in the treatment of pheochromocytoma include:

• The importance of preoperative medical management to stabilize the patient and prevent hemodynamic instability during surgery 4, 5, 3.
• The need for careful selection of alpha-blockers and monitoring of their effects to minimize the risk of complications 5, 6.
• The role of surgical intervention, including laparoscopic and adrenal sparing surgery, in the treatment of pheochromocytoma 7.
• The potential for malignant pheochromocytomas to require palliative treatment options, such as radiotherapy and chemotherapy 7.