Recent developments in the therapy of phaeochromocytoma

Abstract

The basic principles of treatment for phaeochromocytomas and paragangliomas are to block the effects of high catecholamines and make the patient safe for surgical removal of the tumour. The traditional preoperative medical preparation uses the non-selective α-adrenoceptor blocker phenoxybenzamine and a β-adrenoceptor blocker, propranolol. Other agents have been used effectively, including selective α-adrenoceptor blockers, doxazosin and prazosin, and calcium channel antagonists. There have been no trial comparing regimens and there is some controversy as to the best regimen. Major advances have been made in laparoscopic and laparoscopic-assisted surgery. Cortical-sparing adrenalectomy has been used in some centres for familial phaeochromocytomas. High-dose [¹³¹I]-metaiodobenzylguanidine therapy and combined [¹³¹I]-metaiodobenzylguanidine and chemotherapy are promising new developments for the malignant disease. All patients should be followed indefinitely because the recurrence or malignancy rate is ≥ 10% over a prolonged follow up.

• adrenal
• phaeochromocytoma
• paraganglioma
• MIBG