Phaeochromocytoma: Pathology

Key points

• • Phaeochromocytoma arises from the catecholamine-producing chromaffin cells of the adrenal medulla and pours out adrenaline and noradrenaline.
• • The classic picture is episodes of throbbing headache, profuse sweating and pounding palpitations on a background of high blood pressure.
• • A traditional teaching aid calls it a tumour of tens (roughly a tenth are outside the adrenal, on both sides, malignant or familial), but inherited disease is now known to be commoner than this older rule suggests.
• • It can be part of inherited syndromes such as multiple endocrine neoplasia type 2 and von Hippel-Lindau disease.
• • The excess catecholamines and their breakdown products (metanephrines) can be measured in the blood and urine to support the diagnosis.