Recognition and management of phaeochromocytoma

Phaeochromocytomas are potentially lethal catecholamine secreting tumours. 90% occur in the adrenal glands (10% are bilateral) and 10% are extra adrenal. Clinical presentation depends on the pattern of catecholamine secretion. The symptoms are diverse, but the most common are headache, diaphoresis and palpitations. These tumours are important to the anaesthetist because they can present unexpectedly perioperatively and the mortality is up to 50%. Diagnosis relies on the demonstration of excessive catecholamine production by 24 hour urine measurement. The tumour is usually localized by CT or MRI imaging. Excision of the tumour can only be undertaken safely when pharmacological control of the sympathetic nervous system and normovolaemia have been achieved. A number of antihypertensive agents have been used successfully in the perioperative period to maintain cardiovascular stability. Preoperative preparation should also include an assessment of myocardial function to exclude the presence of phaeochromocytoma associated cardiomyopathy. Adrenalectomy can be performed using an open or laparoscopic approach, with each procedure having implications for the anaesthetist. Management of intraoperative hypertensive surges and arrhythmias requires an understanding of the physiology and pharmacology of phaeochromocytomas. This article details the clinical presentation, diagnosis and perioperative anaesthetic management of elective and emergency phaeochromocytomas.