27th May 2025

Management

In benign disease, surgical resection is the first-line treatment. However hypertension must be managed pre-operatively, using the following:

• Alpha-blockers e.g. phenoxybenzamine but this can cause reflex tachycardia due to blockage of alpha-2 receptors. Prazosin and doxazosin are selective alpha-1 antagonists which do not result in reflex tachycardia but do not provide complete alpha blockade.
• Beta-blockers e.g. atenolol, propranolol or metoprolol. These can only be started after sufficient alpha blockade, otherwise it can precipitate hypertensive crisis through unopposed alpha receptor action causing vasoconstriction.
• Calcium channel blocker e.g. nifedipine or amlodipine. These can supplement the above to achieve adequate control of hypertension.

Alpha-blockade can result in volume contraction and orthostatic hypotension, and this can be counteracted by fluid resuscitation and a high salt diet.

In benign disease, surgical methods of resection include:

• Open or laparoscopic total adrenalectomy.
• Adrenal-sparing or partial adrenalectomy. Cortical-sparing adrenalectomy is done in those with hereditary phaeochromocytomas and can avoid need for lifelong corticosteroid replacement, however it requires monitoring for disease recurrence.
• Robot-assisted adrenalectomy.

Malignant tumours can be surgically debulked to provide symptomatic relief, however the mainstay of treatment is CVD (cyclophosphamide, vincristine and dacarbazine) chemotherapy with a complete and partial response rate of up to 57%. Radioactive iodine (iobenguane I-121), external beam radiotherapy and radiofrequency ablation are adjunctive therapies that can be used.