Management of Pheochromocytoma

Diagnosis and Initial Management

• A VMA level of 1500 confirms biochemically active pheochromocytoma and mandates immediate initiation of alpha-adrenergic blockade followed by surgical resection as definitive treatment 1
• Initiate alpha-blockade within 24-48 hours of diagnosis and continue for 10-14 days before any surgical intervention 2, 1
• Target blood pressure: <130/80 mmHg supine AND systolic >90 mmHg standing 2, 1

Diagnostic Imaging

• Obtain CT or MRI of abdomen immediately to localize the tumor and assess for bilateral disease, extra-adrenal paragangliomas, or metastases 3, 4
• Do NOT perform biopsy - this is contraindicated and can precipitate fatal hypertensive crisis 4, 6, 1

Surgical Planning

• Laparoscopic adrenalectomy is the preferred approach for most pheochromocytomas 2, 1
• Complete surgical extirpation (R0 resection) is the only curative treatment 3, 4, 2

Intraoperative Management

• Treat intraoperative hypertension with magnesium sulfate, phentolamine, calcium antagonists, nitroprusside, or nitroglycerin 2
• Anticipate and aggressively treat postoperative hypotension with fluid resuscitation 2, 1

Postoperative Care

• Monitor glucose levels closely - hypoglycemia commonly occurs after catecholamine levels drop 2, 1
• Measure plasma or urine metanephrines at 2-8 weeks to confirm complete tumor removal 2, 1

Long-Term Surveillance

• All patients require lifelong surveillance due to 10-15% recurrence risk 3, 4, 2
• Clinical monitoring (blood pressure, adrenergic symptoms) every 3-4 months for first 2-3 years, then every 6 months 3, 4
• Biochemical testing (plasma/urine metanephrines, chromogranin A) every 3-4 months for 2-3 years, then every 6 months 3, 4