Adrenal Disease - Adrenocortical Carcinoma

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 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.

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.

References

Dackiw APB, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg 2001; 25: 914-926.

Barnett CC, Varma DG, El-Naggar AK, Dackiw APB, Porter GA, Pearson AS, Kudelka AP, Gagel RF, Evans DB, Lee JE. Limitations of size as a criterion in the evaluation of adrenal tumors. Surgery 2000; 128: 973-983.

Gonzalez RJ, Shapiro SE, Sarlis N, Vassilopoulou-Sellin R, Perrier ND, Evans DB, Lee JE. Laparoscopic resection of adrenal cortical carcinoma: A cautionary note. Surgery, 2005 (In Press)