This is a rare cancer that forms in the outer layer of tissue of the adrenal gland, also called adrenocortical cancer and cancer of the adrenal cortex.
Each person has two adrenal glands, which are small and pear-shaped and sit on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.
The adrenal cortex makes important hormones that:
• Balance the water and salt in the body.
• Help keep blood pressure normal.
• Help manage the body's use of protein, fat, and carbohydrates.
• Cause the body to have masculine or feminine characteristics.
The adrenal medulla makes hormones that help the body react to stress.
A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The hormones made by functioning tumors may cause certain signs or symptoms of disease.
Cancer that forms in the adrenal medulla is called pheochromocytoma.
Adrenocortical carcinoma may cause these and other symptoms:
• A lump in the abdomen.
• Pain in the abdomen or back.
A nonfunctioning adrenocortical tumor may not cause symptoms in the early stages.
A functioning adrenocortical tumor makes too much of a certain hormone (cortisol, aldosterone, testosterone, or estrogen).
Too much cortisol may cause:
• Weight gain in the face, neck, and trunk of the body and thin arms and legs.
• Growth of fine hair on the face, upper back, or arms.
• A round, red, full face.
• A lump of fat on the back of the neck.
• A deepening of the voice and swelling of the sex organs or breasts in both males and females.
• Muscle weakness.
• High blood sugar.
• High blood pressure.
Too much aldosterone may cause:
• High blood pressure.
• Muscle weakness or cramps.
• Frequent urination.
• Feeling thirsty.
Too much testosterone (in women) may cause:
• Growth of fine hair on the face, upper back, or arms.
• Acne.
• Balding.
• A deepening of the voice.
• No menstrual periods.
Men who make too much testosterone do not usually have symptoms.
Too much estrogen (in women) may cause:
• Irregular menstrual periods in women who have not gone through menopause.
• Menstrual bleeding in women who have gone through menopause.
Too much estrogen (in men) may cause:
• Growth of breast tissue.
• Lower sex drive.
• Impotence.
Other conditions may cause the same symptoms. Consult a doctor if any of these problems occur.
Different types of treatments are available for patients with adrenocortical carcinoma. Some treatments are standard — often referred to as “standard of care” — and some are being tested in clinical trials. A treatment clinical trial is a research study meant to improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Three types of standard treatment are used:
Surgery
Surgery to remove the adrenal gland (adrenalectomy) is often used to treat adrenocortical carcinoma. Sometimes the nearby lymph nodes are also removed.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Mitotane may be used to treat adrenocortical carcinoma. Mitotane stops the adrenal cortex from making hormones and relieves symptoms caused by the hormones.
