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Adrenocortical Carcinoma

Adrenocortical carcinoma is a malignant tumor that occurs in the cortex, or outer layer of the adrenal gland. It is a very rare cancer, affecting only about 300-500 people annually in the U.S.

About 70% of adrenocortical carcinomas are functioning, meaning that they produce hormones, often in excess amounts. Non-functioning tumors do not produce hormones. Unfortunately, only about 30% of these tumors are confined to the adrenal gland at diagnosis, and have spread to the lung, bone, liver or peritoneum (the membrane lining the abdominal cavity).

Symptoms

Symptoms of adrenocortical carcinoma vary widely, depending on the hormones produced by the tumor. About 50% of tumors overproduce cortisol, causing symptoms of Cushing's syndrome.

Tumors that produce androgen (the "male" hormone) may cause no symptoms at all in men, but women may experience excess growth of facial and body hair, a deepened voice or irregular menstruation.

Tumors that produce estrogen (the "female" hormone) may cause breast tenderness or enlargement, lowered sex drive and/or erectile dysfunction in men, while women may not notice symptoms until the tumor grows large enough to affect nearby organs.

Risk Factors

Age is perhaps the biggest risk factor for adrenocortical carcinoma. Most cases occur in adults between the ages of 40 and 50, although the disease also strikes children under five years of age. Although no genetic basis has been determined, people with Li-Fraumeni syndrome may be at risk. Li-Fraumeni syndrome is an inherited mutation of the p53 "tumor suppressor" gene, which can lead to the development of several different types of cancer, including adrenal tumors.

Read more about Li-Fraumeni syndrome (pdf)

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