Adrenal cancers are rare tumors that may arise in the cortex or medulla of the adrenal gland. It usually occurs in patients 40-50 years of age and is equally distributed between men and women. It may occasionally occur in children less than 5-years old. A cancer arising in the adrenal cortex is called an adrenocortical carcinoma and can produce high blood pressure, weight gain, excess body hair, weakening of the bones and diabetes if hormonally active. A cancer arising in the adrenal medulla is called a pheochromocytoma and can cause high blood pressure, headache, palpitations, and excessive perspiration.
It is unclear what causes adrenal gland cancer, but some cases are associated with hereditary diseases. Some are hormonally active while others are not. More than half of adrenal cancers are functional (produce adrenal hormones). Adrenocortical cancers usually produce cortisol, resulting in Cushing's syndrome. This syndrome causes progressive weight gain, rounding of the face, increased blood pressure, and menstrual cycle alterations in women and feminization in men. Approximately 20% of adrenocortical cancers produce sex hormones (testosterone) causing virilization. Adrenomedullary cancers produce catecholamines, causing pheochromocytoma which is associated with hypertension, weight loss, heart palpitations and anxiety.
For many patients, the diagnosis is made while being evaluated or treated for another condition. As a result, these tumors are often very large when they are diagnosed. Tumors above 4cm are associated with an increased risk of harboring cancer. Many of the tumors are greater than 9cm when they are diagnosed. Evaluation consists of blood and urine for hormonal test and imaging by an abdominal computed tomography scan or magnetic resonance imaging. Imaging usually shows a large, irregular mass with invasion of surrounding structures. When these tumors spread, they do so to lymph nodes, liver and the lungs.
Treatment of adrenal cancer is radical adrenalectomy that is best done with open surgery. This involve removing the adrenal gland and any surrounding structure (when possible) it has invaded. Complete resection can be achieved in up to 70% of cases. Chemotherapy and radiation are sometimes given after surgery. In general, adrenal cancer is associated with a poor outcome. For localized adrenomedullary cancer, the chance of been alive 5-years after surgery can be as high as 95%. Most do not present with localized disease and survival rates at 5-years range from 20% to 45%. For patients with adrenocortical cancer, the chance of been alive 5-years after surgery is 20-60%. If the cancer is not completely removed, the median survival time is less than 1-year.