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Adrenocortical Cancer Overview Adrenal Cancer A rare cancer of the adrenal glands. The usually aggressive cancer causes overproduction of adrenal hormones. This cancer usually occurs in young adults. Signs and Symptoms of Adrenal Cancer Adrenal cancer does not always produce symptoms. Both nonfunctioning adrenocortical carcinomas and large functioning tumors may cause the following: * Fever * Palpable (able to be felt with the fingers) abdominal mass * Persistent abdominal pain * Sensation of abdominal "fullness" * Weight loss Types of adrenal gland tumors A tumor begins when cells become abnormal and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). An adrenal gland tumor can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. An adrenal gland tumor that does not produce hormones is called a nonfunctioning tumor. A tumor can start in an adrenal gland (called a primary adrenal tumor) or it can begin in another organ, such as the lungs, and then metastasize (spread) to the adrenal glands. This section focuses on primary adrenal gland tumors, which include: - * Adenoma. Adenoma is the most common type of adrenal gland tumor, making up 99% of all adrenal gland tumors. It is a noncancerous nonfunctioning tumor of the adrenal cortex. Also called an adrenocortical adenoma, this tumor usually does not cause symptoms, and, if it is small, often does not require any treatment. * Adrenocortical carcinoma. Although very rare, the most common type of cancerous adrenal gland tumor begins in the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Approximately 4 to 12 people out of 1 million develop this type of tumor. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor; if the tumor is functioning, it may produce more than one hormone. * Neuroblastoma. This is a type of childhood cancer that can begin in the adrenal medulla. For more information Risk Factors A risk factor is anything that increases a person’s chance of developing a tumor. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of a tumor, most do not directly cause the tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health care choices. The cause of most cases of cancerous adrenal cortical carcinoma is not known. However, people with certain hereditary conditions have a higher risk of developing an adrenal gland tumor, including Li-Fraumeni syndrome and Carney complex. People who have a high risk of developing an adrenal gland tumor because of family history should be examined and evaluated yearly by their doctor. Symptoms People with an adrenal gland tumor may experience the following symptoms. Sometimes, people with an adrenal gland tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom on this list, please talk with your doctor. * High blood pressure * Low potassium level * Heart palpitations * Nervousness * Anxiety (panic attacks) * Headache * Excessive perspiration * Diabetes * Abdominal pain * Unexplained weight gain * Unexplained weight loss * Weakness * Abdominal stretch marks * Excessive hair growth * Unusual acne * Change in libido (sex drive) Diagnosis Doctors use many tests to diagnose a tumor and determine if it has metastasized. Some tests may also determine which treatments may be the most effective. To diagnose an adrenal gland tumor, blood and urine tests (see below) are done to look for certain substances that may indicate that cancer is present. If the doctor suspects that the cancer has spread from another area of the body, a biopsy may be performed to determine the type of cancer. In addition to a physical examination, the following tests may be used to diagnose an adrenal gland tumor: - Blood and urine tests. Blood tests can measure the amounts of natural hormones produced during stress, such as catecholamines and metanephrines, which can detect a functional tumor. A patient may be asked to take a pill on the evening before the blood and urine tests, which helps detect the normal suppression of production of the hormone cortisol. A 24-hour urine sample, which requires the collection of all urine during that timeframe for laboratory testing, may also be necessary. This helps the doctor track how quickly various hormones are produced. Tell your doctor about any medications that you take, even over-the-counter drugs, because this information is needed to correctly interpret the results. Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. For an adrenal tumor, a hollow needle is used to collect the tissue. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail. Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium is injected into a patient’s vein to provide better detail. Metaiodobenzylguanidine (MIBG) scan. MIBG is a chemical similar to adrenaline that will collect in a neuroendocrine tumor. A MIBG scan can show a tumor of the adrenal medulla that may not appear in an x-ray. The scan takes place over two consecutive days. On the first day, an injection of MIBG is given in the arm. Several hours later, pictures are taken with a special camera that can detect if or where in the body the MIBG has collected. The following morning, more pictures are taken, and the process may be repeated if necessary. Treatment The treatment of an adrenal gland tumor depends on the size and location of the tumor, if it is cancerous, whether the cancer has spread, and the patient’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Mitotane (Lysodren) is a medication that is used to treat cancer that affects the adrenal cortex. It reduces the amount of adrenocorticoids produced by the adrenal cortex. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Radiation therapy Radiation therapy is the use of high energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. 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