Operative Management of Bilateral Pheochromocytoma

Preoperative Management

The optimal preoperative management of bilateral pheochromocytoma requires alpha-adrenergic blockade for 10-14 days, with blood pressure targets of <130/80 mmHg supine and >90 mmHg systolic when standing, according to the American College of Cardiology and the European Society of Endocrinology 1, 2
Alpha-adrenergic blockade must be initiated at least 7-14 days before surgery with gradually increasing dosages, as recommended by the European Society of Endocrinology 1, 3
Both selective α1-blockers (doxazosin, prazosin, terazosin) and non-selective blockers (phenoxybenzamine) are effective, with phenoxybenzamine potentially providing less intraoperative hemodynamic instability, according to the European Society of Endocrinology 3
Beta-blockers should only be added after adequate alpha blockade to control tachyarrhythmias, never before alpha blockade, as recommended by the American College of Cardiology 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, according to the European Society of Endocrinology 3
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, as recommended by the European Society of Endocrinology 3

In patients with multifocal disease, functional pheochromocytomas should be resected as an initial priority due to the risk of perioperative hypertensive crisis, according to the European Society of Endocrinology 2
For patients with hereditary syndromes or high risk of metachronous tumors, careful consideration of cortical-sparing techniques versus total adrenalectomy must be made, as recommended by the European Society of Endocrinology 2
Laparoscopic adrenalectomy is the preferred approach for most bilateral pheochromocytomas, showing better intraoperative hemodynamic stability compared to open surgery, according to the European Society of Endocrinology 2
Open surgery should be considered for tumors with high suspicion of malignancy, large size (>6 cm), or local invasion, as recommended by the European Society of Endocrinology 2
Complete surgical extirpation (R0 resection) is the mainstay of potentially curative approaches, according to the American College of Surgeons 4

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

Careful monitoring of glucose levels is essential as hypoglycemia may occur after reduction of catecholamine levels, according to the American College of Endocrinology 1
Postoperative biochemical testing should be performed 2-8 weeks after surgery for functional tumors to confirm complete resection, as recommended by the European Society of Endocrinology 5
Imaging should be done at 3-6 months postoperatively to verify complete tumor removal, according to the European Society of Endocrinology 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, as recommended by the European Society of Endocrinology 2
Cytoreductive (R2) resection may improve quality of life and survival in malignant pheochromocytoma by reducing tumor burden and controlling hormonal hypersecretion, according to the American College of Surgeons 1

Patients with resected pheochromocytoma should be followed at regular intervals with clinical, imaging, and biochemical screens for at least 10 years, as recommended by the American College of Endocrinology 4
For functional tumors, measurement of plasma or urine metanephrines should be performed by 8 weeks post-treatment, according to the European Society of Endocrinology 5
Imaging should be done at 3-6 months post-treatment and then periodically based on risk stratification, as recommended by the European Society of Endocrinology 5
Lifelong surveillance with increased intervals is recommended for patients with malignant or hereditary pheochromocytoma, according to the American College of Endocrinology 4