1. Rare But Deadly
Adrenocortical carcinoma is not a phrase many people are apt to hear too often; this rare type of cancer is seen in only one out of 1.5 million people. It affects the outer layer (adrenal cortex) of the adrenal glands, which are small glands found on top of each kidney. The adrenal glands produce hormones that help to regulate blood pressure, heart rate, salt and water balance and management of fats, proteins and carbohydrates. Tumors of the adrenal cortex can be either functioning (when too many hormones are produced) or non-functioning (production of hormones has been stopped).
2. Too Much of These Hormones Can Be a Bad Thing
In the early stages of adrenocortical carcinoma, a non-functioning tumor will likely result in no appearance of symptoms. A functioning tumor, however, will cause the overproduction of these hormones: estrogen, aldosterone, testosterone and cortisol. Too much of these hormones can result in weight gain in the face and torso, fine hair growth on the face, upper back and arms, high blood pressure, elevated blood sugar, frequent thirst and frequent urination. Too much estrogen in women can cause irregular menstrual cycles, or menstrual bleeding in post-menopausal women and growth of breast tissue, decreased sex drive and impotence in men.
3. As Adrenocortical Carcinoma Grows
As in most cancers, there are stages of adrenocortical carcinoma. In Stages I and II, the tumor is either under five centimeters (I) or over five centimeters (II), but is in both stages contained to the adrenal gland. Stage III disease is when the tumor can be any size and has spread to nearby fat or lymph nodes. The fourth stage is when other organs are involved, including lungs, liver, bones and the connective tissue in the abdomen. Recurrent adrenocortical carcinoma is when the disease has been treated and then returns, either in the adrenal gland or other organs.
4. Tests, Tests and More Tests
Blood and urine tests and imaging of the abdominal area are methods by which adrenocortical carcinoma is determined. A 24-hour test of the urine can be used to discover an elevated amount of cortisol in the system. Certain blood tests will show higher amounts of estrogen or testosterone as well as cortisol. A study of the blood chemistry may be performed. A CT scan or MRI will show the adrenal cortex and any tumors present. A PET (positron emission tomography) scan also shows the presence of malignant tumors in the body.
5. Fighting a Tough Opponent
Treatment for this type of cancer varies according to the stage the disease is in when treatment begins. Oral mitotane (drug name: Lysodren) is used in most stages and in recurrent disease for functioning tumors. Surgical removal of the adrenal glands and even nearby lymph nodes is sometimes used to combat adrenocortical carcinoma. Radiation therapy and chemotherapy are also options depending on the stage of the disease. New biologic therapies to use the immune system to fight the cancer are in clinical study.


