Management of phaeochromocytoma

For management of phaeochromocytoma in children, seek specialist advice.

Phaeochromocytoma in adults is treated by surgical removal. Once the diagnosis is confirmed, start drug therapy to control blood pressure and to ensure alpha-adrenergic blockade before surgery. This minimises the risk of a hypertensive crisis during surgery, as well as postoperative hypotension. Drug therapy is also appropriate if surgery is contraindicated. For management of a hypertensive crisis associated with phaeochromocytoma, see here.

Treatment as an outpatient for at least 2 weeks is usually required to achieve adequate preoperative control. Phenoxybenzamine, a noncompetitive alpha-adrenergic blocker, has traditionally been preferred to competitive blockers such as prazosin, which can be displaced from alpha receptors by a rise in concentration of endogenous catecholamines. However, some studies have shown similar perioperative outcomes with either class of drug. Use:

1 phenoxybenzamine 10 mg orally, twice daily initially. Increase the dose by 10 mg every 2 to 3 days to a target blood pressure of 120/80 mmHg with 20/10 mmHg postural drop. Usual daily dose 1 mg/kg in 2 or 3 divided doses phaeochromocytoma phenoxybenzamine    

OR

2 prazosin 0.5 mg orally, twice daily initially. Gradually increase the dose to 2 to 5 mg, 2 or 3 times daily. Increase the dose to a target blood pressure of 120/80 mmHg with 20/10 mmHg postural drop. phaeochromocytoma prazosin    

Alpha-adrenergic blockade can be considered adequate when:

  • episodic symptoms resolve
  • blood pressure reduces to the desired target
  • postural drop is at least 20/10 mmHg (major symptomatic postural hypotension indicates overtreatment).

Reflex tachycardia is common with alpha-adrenergic blockade, and a beta blocker is usually required to control heart rate. Do not start a beta blocker before establishing alpha blockade. Use of a beta blocker in a patient without adequate alpha-adrenergic blockade is contraindicated in a patient with phaeochromocytoma; unopposed alpha receptor–mediated vasoconstriction can cause an extreme rise in blood pressure.

Note: Do not start beta blockade in a patient with phaeochromocytoma until alpha blockade has been established.

If beta-blocker therapy is required, use:

1 atenolol 50 mg orally, twice daily initially. Increase the dose as required to a target heart rate of 60 beats per minute. Maximum dose 100 mg twice daily phaeochromocytoma atenolol    

OR

1 bisoprolol 2.5 mg orally, twice daily initially. Increase the dose as required to a target heart rate of 60 beats per minute. Maximum dose 5 mg twice daily phaeochromocytoma bisoprolol    

OR

1 metoprolol 50 mg orally, twice daily initially. Increase the dose as required to a target heart rate of 60 beats per minute. Maximum dose 100 mg twice daily. phaeochromocytoma metoprolol