Perioperative care of phaeochromocytoma

Abstract

### Key points Phaeochromocytomas are catecholamine-secreting tumours of the adrenal medulla, while paragangliomas are closely related neuroendocrine tumours arising from extra-adrenal paraganglia, some of which produce catecholamines. In this article, their perioperative management will be considered together. The majority develop sporadically, although around one-third of cases have specific gene mutations which are usually inherited in an autosomal-dominant fashion. These may be associated with other tumours, for example, multiple endocrine neoplasia 2A and 2B, Von Hippel–Lindau disease, succinate dehydrogenase enzyme mutations, and neurofibromatosis. The annual European incidence rate of phaeochromocytomas is around 0.2 per 100 000 people. The traditional ‘Rule of 10s’ states that 10% of phaeochromocytomas are ‘extra-adrenal’, 10% are malignant, 10% are bilateral, 10% are found in normotensive patients, and 10% are familial. However, this statement probably no longer holds true as there appears to be a significantly higher proportion of tumours that are malignant (29%),1 extra-adrenal (24%),2 and/or familial (32%).3 Phaeochromocytomas may present with a classic symptom triad of headache, palpitations, and sweating. Hypertension is present in around 90% of cases, although it is paroxysmal in 35–50% of these. Other non-specific presentations include anxiety, lethargy, nausea, weight loss, hyperglycaemia, and tremor. Abdominal pain may result from bowel ischaemia due to excessive vasoconstriction. Visual disturbance may develop from papilloedema induced by malignant hypertension. Half of phaeochromocytomas are diagnosed incidentally on abdominal imaging for an unrelated indication. Phaeochromocytomas produce a variable mixture of norepinephrine, epinephrine, or, more rarely, dopamine. Traditional biochemical diagnosis of phaeochromocytomas relied upon 24 h collections …