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Overview. Adrenal cancer is an extremely rare but potentially deadly malignancy that starts in the adrenal glands, most often causing hormone overproduction. The disease is diagnosed in approximately 1-2 people per 1 million population each year, most commonly in young adults or in children under the age of 6. Although it can be cured when found in its early stages, more than two-thirds of the time the tumor has spread beyond the adrenal gland by the time it is discovered, dampening the prognosis. When the tumor is found at the early stage and can be removed surgically, the five-year survival rate is 50-60 percent. The prognosis for adrenal cancers that have spread to nearby or distant organs is much less favorable, with only 10-20 percent surviving five years.
Risk Factors. It is unclear what causes adrenal cancer or what puts people at higher risk, although in a minority of instances genetic factors can come into play. Mutations in tumor suppressor genes, including Li-Fraumeni syndrome and type 1 multiple endocrine neoplasia (MEN1), increase the risk for adrenal and other cancers. Other familial syndromes associated with a higher risk for the disease include Gardner syndrome, Carney triad, Cowden syndrome, familial polyposis and Turcot syndrome.
Anatomy. Located in the upper abdomen above the kidneys, the adrenal glands consist of the cortex – the outer layer that produces corticosteroid hormones such as testosterone; and the medulla – the central core responsible for producing epinephrine, norepinephrine and dopamine, the adrenaline hormones that are activated in the so-called “fight-of-flight” response.
Symptoms and Tumor Types. The vast majority of adrenal tumors are benign. Most often found incidentally during diagnostic imaging, they typically cause no symptoms and require no treatment. Among the cancerous tumors, there are two types: functioning and non-functioning. The more-common functioning adrenal tumors increase hormone production and produce symptoms related to the hormones that are overproduced. These may include abnormal weight gain or weight loss, hypertension, and anxiety. Women may see altered menstrual cycles and men may experience feminization, while children may experience early puberty. Non-functioning adrenal tumors fail to produce hormones and can produce pain from pressure on the abdominal organs, as well as a mass in the abdomen that can be felt with the fingers.
Functioning adrenal tumors that originate in the cortex – the most common form of adrenal cancer – produce excess cortisol that can result in Cushing’s syndrome, whose symptoms include rapid weight gain, rounding of the face, excessive sweating, easy bruising and excess facial or body hair growth in women.
Symptoms resulting from excess hormone production or from the abdominal pain that comes with non-functioning tumors are what most often prompt patients to seek medical attention that leads to the tumor’s discovery.