What is the operative management of bilateral pheochromocytoma?

Operative Management of Bilateral Pheochromocytoma

The optimal operative management of bilateral pheochromocytoma requires preoperative alpha-adrenergic blockade for 10-14 days, followed by staged or simultaneous bilateral adrenalectomy, with laparoscopic approach being preferred for most cases. 1

Preoperative Management

• Alpha-adrenergic blockade must be initiated at least 7-14 days before surgery with gradually increasing dosages until blood pressure targets (<130/80 mmHg supine, >90 mmHg systolic when standing) are achieved 1
• Both selective α1-blockers (doxazosin, prazosin, terazosin) and non-selective blockers (phenoxybenzamine) are effective, with phenoxybenzamine potentially providing less intraoperative hemodynamic instability 1
• Beta-blockers should only be added after adequate alpha blockade to control tachyarrhythmias, never before alpha blockade 1
• Calcium channel blockers may be used as adjuncts to alpha-blockers for refractory hypertension or as monotherapy in cases with normal to mildly elevated blood pressure 1
• High-sodium diet and administration of 1-2 liters of saline 24 hours prior to surgery, along with compressive stockings, should be employed to reduce the risk of orthostatic and postoperative hypotension 1

Surgical Approach

Timing and Sequencing

• For bilateral pheochromocytomas, surgery can be performed either as a single procedure or in two stages 2
• In patients with multifocal disease, functional pheochromocytomas should be resected as an initial priority due to the risk of perioperative hypertensive crisis 1
• For patients with hereditary syndromes or high risk of metachronous tumors, careful consideration of cortical-sparing techniques versus total adrenalectomy must be made 1

Surgical Technique

• Laparoscopic adrenalectomy is the preferred approach for most bilateral pheochromocytomas, showing better intraoperative hemodynamic stability compared to open surgery 3
• The technical approach can be either anterior or posterior (retroperitoneoscopic) depending on surgical expertise 1
• Open surgery should be considered for tumors with high suspicion of malignancy, large size (>6 cm), or local invasion 1
• Complete surgical extirpation (R0 resection) is the mainstay of potentially curative approaches 1

Intraoperative Management

• Hypertension during surgery may be treated with magnesium sulfate, intravenous α-adrenoreceptor antagonist (phentolamine), calcium antagonists, nitroprusside, or nitroglycerin 1
• Tachycardia can be treated with intravenous β-adrenergic receptor blocker (esmolol) 1
• Postoperative hypotension should be prevented by adequate preoperative saline infusion and treated aggressively if it occurs 1

Postoperative Care

• All patients should be monitored in an intensive care setting for at least 24-48 hours postoperatively 4
• Careful monitoring of glucose levels is essential as hypoglycemia may occur after reduction of catecholamine levels 1
• Patients who undergo bilateral adrenalectomy will require lifelong glucocorticoid replacement (typically hydrocortisone 30 mg daily) 2
• Postoperative biochemical testing should be performed 2-8 weeks after surgery for functional tumors to confirm complete resection 1
• Imaging should be done at 3-6 months postoperatively to verify complete tumor removal 1

Special Considerations

• In patients with hereditary syndromes (MEN, VHL, SDHx mutations), genetic testing and screening of family members is recommended 5
• For patients with high risk of metachronous disease, cortical-sparing techniques may be considered, but must be weighed against the risk of incomplete tumor removal due to multifocality 1
• Cytoreductive (R2) resection may improve quality of life and survival in malignant pheochromocytoma by reducing tumor burden and controlling hormonal hypersecretion 1

Follow-up Protocol

• Patients with resected pheochromocytoma should be followed at regular intervals with clinical, imaging, and biochemical screens for at least 10 years 1
• For functional tumors, measurement of plasma or urine metanephrines should be performed by 8 weeks post-treatment 1
• Imaging should be done at 3-6 months post-treatment and then periodically based on risk stratification 1
• Lifelong surveillance with increased intervals is recommended for patients with malignant or hereditary pheochromocytoma 1