8 Phaeochromocytoma

Phaeochromocytomas are uncommon among patients with hypertension, and sometimes occur in persons without known hypertension, but are important to detect because they are often lethal but commonly curable, and because they are a clue to the presence of associated conditions. Paroxysmal symptoms (especially headache, palpitations, diaphoresis and anxiety), hypertension that is intermittent, unusually labile or resistant to conventional therapy, and conditions known to be associated raise the clinical suspicion of phaeochromocytoma. Biochemical confirmation is commonly achieved by measurement of urinary catecholamines, metanephrines or VMA. Plasma noradrenaline and adrenaline measure-ments may be superior to measurements of urinary catecholamine metabolites, but strict attention to the details of sample collection, handling and storage, the many sources of possible biological variation and the effects of drugs is critical if diagnostic error is to be avoided. Patients should be evaluated in the drug-free state if at all possible. Anatomical localization, in the abdomen in the vast majority of cases and usually in the adrenal medullae, can generally be accomplished with computed tomographic scans. Bilateral adrenomedullary tumours are the rule in familial phaeochromocytoma. Most phaeochromocytomas are benign and can be excised totally after medical preparation with an α-adrenergic antagonist.