How is pheochromocytoma managed?

Management of Pheochromocytoma

Complete surgical resection is the mainstay of treatment for pheochromocytoma, with mandatory preoperative alpha-adrenergic blockade for patients with norepinephrine-producing tumors to prevent potentially fatal perioperative complications. 1

Diagnosis

Before treatment, proper diagnosis is essential:

• Best screening test: plasma free metanephrines (normetanephrine and metanephrine) with 99% sensitivity and 89% specificity 1
• Clinical presentation typically includes:
  • Episodic headaches, sweating, and palpitations (90% diagnostic specificity) 1
  • Sustained or paroxysmal hypertension (occurs in ~70% of cases) 1
  • Blood pressure variability (related to norepinephrine secretion levels) 1

Preoperative Management

Alpha-Adrenergic Blockade

Alpha-adrenergic blockade is mandatory before any surgical intervention for norepinephrine-producing pheochromocytomas 1:

• Start 7-14 days before surgery 1
• Options include:
  • Selective α1-blockers: doxazosin, prazosin, or terazosin
  • Non-selective α-blocker: phenoxybenzamine (FDA-approved specifically for pheochromocytoma) 2
• Phenoxybenzamine may provide slightly better control of systolic blood pressure but causes more pronounced postoperative hypotension 3
• Target blood pressure: <130/80 mmHg when supine, systolic >90 mmHg when standing 1

Beta-Adrenergic Blockade

• Only add after adequate alpha-blockade is established 1, 2
• Indicated for tachycardia and catecholamine-induced arrhythmias 4
• Never use beta-blockers alone as monotherapy (can precipitate hypertensive crisis) 1

Additional Measures

• High-sodium diet and 1-2 liters of saline 24 hours before surgery 1
• Compressive stockings to reduce orthostatic hypotension 1
• Consider metyrosine (inhibits catecholamine synthesis) as an add-on where available 1, 4
• Calcium channel blockers can be used as adjuncts for refractory hypertension 1

Surgical Management

• Surgery is the definitive treatment for pheochromocytoma 1

• Surgical approach:

  • Laparoscopic adrenalectomy is safe and effective for pheochromocytomas 1
  • Open surgery with transperitoneal access for larger or potentially invasive tumors 1
  • Surgery should be performed in specialized centers with experience in adrenal surgery 1

• For malignant pheochromocytoma, cytoreductive (R2) surgery may improve quality of life and survival by reducing tumor burden and controlling hormonal hypersecretion 1

Special Situations

Pheochromocytoma in Pregnancy

• If diagnosed within first 24 weeks: laparoscopic adrenalectomy after 10-14 days of alpha-blockade 1
• If diagnosed in third trimester: medical management until fetus is viable, then cesarean section with tumor removal in the same session 1
• Untreated maternal and fetal mortality is ~50%, but early detection and treatment reduces this to <5% maternal and 15% fetal mortality 1

Hypertensive Crisis Management

• Intravenous alpha-1-blockers (phentolamine, doxazosin, terazosin) or labetalol 1
• Sodium nitroprusside may be needed for severe hypertension during tumor manipulation 5

Follow-up

• Long-term follow-up is essential for at least 10 years 1
• For malignant pheochromocytoma, lifelong surveillance is recommended 1
• Follow-up schedule:
  • Every 3-4 months for 2-3 years
  • Every 6 months thereafter 1
• Monitoring includes:
  • Clinical evaluation (adrenergic symptoms and blood pressure)
  • Biochemical testing (plasma/urinary metanephrines, normetanephrines, chromogranin A)
  • Imaging for patients with high risk of recurrence or metastasis 1

Pitfalls to Avoid

1. Never use beta-blockers before establishing adequate alpha-blockade (can precipitate hypertensive crisis) 1
2. Don't underestimate the importance of preoperative preparation - inadequate blockade can lead to fatal complications during surgery 6
3. Be aware of postoperative hypotension, especially with phenoxybenzamine 3
4. Remember that diagnosis is often delayed (average 3 years from initial symptoms) 1
5. Consider genetic testing as up to 25% of pheochromocytomas are hereditary 7