Management of Pheochromocytoma
Definitive Treatment
Surgical resection via laparoscopic adrenalectomy is the only curative treatment for pheochromocytoma and should be performed after 10-14 days of preoperative alpha-adrenergic blockade. 1, 2, 3
Preoperative Medical Preparation
Alpha-Adrenergic Blockade (MANDATORY)
- Initiate alpha-blockade immediately upon biochemical confirmation and continue for 10-14 days before surgery 1, 2, 3
- Target blood pressure: <130/80 mmHg supine AND systolic >90 mmHg when standing 1, 2, 3
Choice of Alpha-Blocker:
- Phenoxybenzamine (non-selective, non-competitive): FDA-approved for pheochromocytoma; may provide superior intraoperative hemodynamic stability and better alpha-blockade 4, 5
- Selective alpha-1 blockers (doxazosin, prazosin, terazosin): Equally effective alternatives with fewer side effects (less orthostatic hypotension, edema, nasal congestion) but may require more additional antihypertensive agents 6, 1, 7
The 2024 ESC guidelines note that both phenoxybenzamine and selective alpha-1 blockers are effective, with phenoxybenzamine potentially providing less intraoperative hemodynamic instability 6. However, recent research shows phenoxybenzamine costs significantly more ($442/day vs $5/day for doxazosin) without clear superiority in preventing cardiovascular complications 5.
Beta-Blockade (ONLY AFTER Alpha-Blockade)
- NEVER initiate beta-blockers before adequate alpha-blockade—this can precipitate hypertensive crisis from unopposed alpha-adrenergic stimulation 1, 3
- Add beta-blockers only after alpha-blockade is established to control reflex tachycardia and tachyarrhythmias 6, 1, 3
Adjunctive Therapies
- Calcium channel blockers: Use as adjuncts for refractory hypertension or as monotherapy in normotensive/mildly hypertensive patients 6, 1, 3
- Metyrosine (alpha-methyltyrosine): Catecholamine synthesis inhibitor that may improve intraoperative blood pressure control, reduce blood loss, and decrease fluid requirements when combined with phenoxybenzamine 8, 9
Volume Expansion
- High-sodium diet and 1-2 liters of saline 24 hours before surgery, plus compression stockings, to prevent orthostatic and postoperative hypotension 1, 3
- Phenoxybenzamine induces significant plasma volume expansion (+14.5%) which counteracts the peripheral hypovolemia characteristic of pheochromocytoma 10
Surgical Approach
Technique Selection
- Laparoscopic adrenalectomy is preferred for most pheochromocytomas 6, 1, 2, 3
- Open surgery for: tumors >6 cm, high suspicion of malignancy, or local invasion 1, 3
- Complete R0 resection is the only potentially curative approach 1, 2, 3
Bilateral Disease Considerations
- Resect functional tumors first due to perioperative hypertensive crisis risk 1
- Consider cortical-sparing techniques in hereditary syndromes to preserve adrenal function, but weigh against risk of incomplete resection 1
Intraoperative Management
Hypertensive Crisis Treatment
- Phentolamine (IV alpha-1 blocker): First-line for adrenergic crises 6, 1
- Alternatives: Magnesium sulfate, calcium antagonists, nitroprusside, nitroglycerin 6, 1, 2
- Labetalol (IV): 1-2 mg/kg bolus followed by continuous infusion; provides both alpha and beta blockade, avoiding tachycardia 6
Tachycardia Management
Hypotension Management
- Aggressive fluid resuscitation for postoperative hypotension 1, 2, 3
- Anticipate hypotension after tumor removal due to sudden catecholamine withdrawal and peripheral hypovolemia 6
Postoperative Care
Immediate Monitoring
- Monitor glucose levels closely—hypoglycemia commonly occurs after catecholamine levels drop 1, 2, 3
- Aggressive treatment of hypotension with fluids 1, 2
Biochemical Confirmation
- Measure plasma or urine metanephrines at 2-8 weeks post-surgery to confirm complete tumor removal 1, 2, 3
- Imaging at 3-6 months to verify complete resection 1
Long-Term Surveillance
Follow-Up Protocol
- Lifelong surveillance required due to 10-15% recurrence risk 2
- Clinical monitoring (blood pressure, adrenergic symptoms): Every 3-4 months for 2-3 years, then every 6 months 2
- Biochemical testing (plasma/urine metanephrines, chromogranin A): Every 3-4 months for 2-3 years, then every 6 months 2
- Imaging: At 3-6 months post-treatment, then periodically based on risk stratification 1
- Minimum 10 years of surveillance for all patients 1
Critical Pitfalls to Avoid
- NEVER perform fine needle biopsy of suspected pheochromocytoma—can precipitate fatal hypertensive crisis 2, 3
- NEVER start beta-blockers before alpha-blockade 1, 3
- Do not proceed to surgery without adequate preoperative preparation (10-14 days of alpha-blockade) 1, 2, 3
- Do not assume cure without biochemical confirmation at 2-8 weeks post-surgery 1, 2, 3