Steroid-secreting human adrenocortical tumours are rare. Their incidence is estimated to be 0.2/100 000 per year (1). Benign adenomas and the malignant carcinomas are about equally frequent (2–4). The most common clinical presentation is that of Cushing's syndrome with or without association virilism. Adenomas responsible for primary aldosteronism (Conn's syndrome) are less frequent, and oestrogen-producing tumours—usually malignant— are rare (5). Approximately half the malignant adrenocortical cancers secrete mainly biosynthetic precursors with diminished bioactivity: their diagnosis is often delayed and they may thus be huge when discovered. More often sporadic, adrenocortical tumours can also combine with rare congenital syndromes including Beckwith-Wiedemann syndrome (6), the McCune-Albright syndrome (7) or Li-Fraumeni syndrome (8).
Because there are no absolute clinical, biological, anatomical or even histological criteria, in many cases the benign or malignant nature of a localized tumour cannot be strictly asserted. Malignant adrenocortical tumours have a very poor prognosis (2–4, 9–15): mean survival
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