Adrenocortical carcinoma in the McMaster Textbook of Internal Medicine

Adrenocortical carcinoma (ACC) is a rare epithelial malignancy originating in the adrenal cortex, prone to local invasion and distant metastases. Steroidogenesis disorders are quite common in this condition. Tumors may overproduce mainly cortisol and androgens but sometimes also estrogens and mineralocorticoids. The tumor’s ability to synthesize biologically active glucocorticoids depends on its cellular differentiation; less differentiated tumors are less likely to be functioning. Signs or symptoms of increased hormonal activity may be absent.

“The peak incidence of ACC usually has a bimodal age distribution, with the disease peaking at <5 years of age and in the fourth to fifth decade of life. ACC is more common in women and, although sporadic in the majority of cases, it can occur as part of hereditary syndromes such as Li-Fraumeni syndrome, Carney complex, Lynch syndrome, multiple endocrine neoplasia type 1 (MEN 1), and familial adenomatous polyposis. Clinical features and natural history depend on whether the tumor is hormonally active (functioning, 50%-60%) or nonfunctioning.”

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