What is Adrenal Cortical Carcinoma?
Adrenal cortical carcinoma (ACC) is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.There are two adrenal glands. The adrenal glands are small and shaped like a triangle. Adrenal cortical carcinoma (ACC) is caused by a cancerous growth in the adrenal cortex, which is the outer layer of the adrenal glands. The adrenal glands lie on top of the kidneys. They play an important role in the endocrine system, which is the system that produces and regulates hormones.
The adrenal cortex makes hormones that regulate metabolism and blood pressure. It also produces cortisol and the male hormones called androgens, such as testosterone. ACC may trigger excessive production of these hormones.
Types of Adrenal Cortical Carcinomas
There are two types of adrenal cortical carcinomas.
Functioning tumors increase the production of adrenal hormones. With this type of tumor, large amounts of cortisol, testosterone, and aldosterone are usually found in the body. (Aldosterone is a hormone that regulates blood pressure.)
Nonfunctioning tumors do not increase the adrenal glands’ hormonal production.
Most tumors on the adrenal glands are not cancerous. Only 5 to 10 percent of adrenal tumors are malignant.
Symptoms of an Adrenal Cortical Carcinoma?
The symptoms of a functioning tumor depend on which hormones it is producing.
Testosterone and other androgens:
- increased facial and body hair, particularly in females
- deepened voice in females
- early signs of puberty in children
- enlarged breast tissue in males
- weight gain
- high blood pressure
- high blood sugar and pressure
- muscle weakness in the legs
- bruising in the body
- excessive weight gain in the chest and abdomen
Both functioning and nonfunctioning tumors can cause abdominal pain if they become enlarged. Nonfunctioning tumors may not produce any hormonal changes or cause specific symptoms
Cushing’s syndrome is a condition caused by cortisol producing adrenal tumors. Although
Causes of Adrenal Cortical Carcinoma
The causes of primary Adrenal Cortical Carcinoma are unknown. However, Adrenal Cortical Carcinoma can also be a secondary cancer. A secondary cancer is what happens when another form of cancer spreads to the adrenal glands.
Risk factor for Adrenal Cortical Carcinoma?
Scientists have identified a number of risk factors for Adrenal Cortical Carcinoma. You may be at higher risk if you:
- have a hereditary disease that affects the adrenal glands
- have another form of cancer that is aggressive
- are female
- are between the ages of 40 and 50
Children under age 5 are also at higher risk for this condition. Keep in mind, Adrenal Cortical Carcinoma is a rare cancer. Just because you have one or more risk factors does not mean that you will get Adrenal Cortical Carcinoma.
Diagnosis of Adrenal Cortical Carcinoma
To diagnose Adrenal Cortical Carcinoma your doctor will perform a physical exam. You may also need lab tests to check your hormone levels. This could require collecting your saliva, blood, and urine.
Your doctor might also want to use imaging tests to look for a tumor on your adrenal glands. These tests may include:
- magnetic resonance imaging (MRI)
- computed tomography (CT) scan
- positron emission tomography (PET) scan
If a tumor is found, a small piece of tissue might be taken for study. This is called a biopsy. A biopsy allows your doctor to see if tumor cells are cancerous or benign. Most adrenal tumors are non-cancerous.
Treatment of Adrenal Cortical Carcinoma
Your doctor will develop a treatment plan based on your condition, sex, age and overall physical health. The doctor may also stage your cancer. Staging tells your doctor how advanced your cancer is and can help determine the right treatment.
Tumor stages are defined as follows:
- Stage 1 tumors: They are small tumors (less than 5 centimeters) that are still within the tissues.
- Stage 2 tumors are: They large tumors (greater than 5 centimeters) that are still within the tissues.
- Stage 3 tumors: They are tumors of any size that have spread to nearby lymph nodes and fatty tissue.
- Stage 4 tumors: They are tumors of any size that have spread to other organs and tissues.
Depending on the stage of your cancer, a variety of treatments are available.
Chemotherapy uses drugs to kill cancer cells in the body. These drugs may be taken by mouth or administered through the veins.
Surgery can remove the adrenal gland and surrounding tissue, if necessary.
Radiation can be used to kill cancer cells. There are two types of radiation therapy. External radiation therapy is applied from outside your body. Internal therapy applies radioactive substances directly to the tumor, inside your body. Catheters, needles, or wires may be used to administer internal therapy.
Biologic therapy uses your own immune system and body to destroy the cancer.
Outlook for Adrenal Cortical Carcinoma
The stage of your cancer may influence how well treatments work. Your doctor will request follow-up visits in order to check your cancer’s response to treatment. Sometimes a tumor may return, and you may need further treatment.
Your doctor will also monitor your health for other potential problems related to Adrenal Cortical Carcinoma. The hormone changes caused by functional tumors can lead to additional symptoms. The course of treatment you receive may focus on helping to resolve these issues.