Incidence

Functioning adrenocortical neoplasms with clinical manifestations of hypersecretion occur in 4 cases per million and roughly half are adenomas and the rest carcinomas. Adrenocortical carcinomas at autopsy account for 2.5 cases per million. Hence, the suggested incidence of nonfunctioning adrenocortical carcinomas should be 0.6 to 1.7 cases per million.3

If these figures are matched with the prevalence of adrenal masses found incidentally (i.e., 0.6% to 1.3% of the ambulatory population), it is evident that, in the setting of the adrenal "incidentaloma," nonfunctioning adrenocortical carcinomas are an uncommon cause. Van Heerden and colleagues4 reported that only 4 of 342 (1.2%) patients with incidentalomas at the Mayo Clinic had adrenocortical cancers. Our experience in Lille, France, of adrenal incidentalomas is consistent with this finding. Among 213 adrenal incidentalomas, of which 103 were operated, there were 5 (2%) adrenocortical carcinomas.5 Pheochromocytomas and metastasis to the adrenal gland should be the primary concern in this setting, because they occur in 1 % to 15% and 4% to 22%, respectively.6

The characteristics of 54 adrenocortical carcinomas, among 486 patients who underwent adrenal surgery, are listed in Table 69-1.

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