What Is Adrenal Cortical Carcinoma?
Adrenal cortical carcinoma (ACC) is a rare disease. It 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. ACC is also known as adrenocortical carcinoma.
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.
What Causes an Adrenal Cortical Carcinoma?
The causes of primary ACC are unknown. However, ACC can also be a
secondary cancer. A secondary cancer is what happens when another form of
cancer spreads to the adrenal glands.
Who Is at Risk for an Adrenal Cortical
Scientists have identified a number of risk factors for ACC. You
may be at higher risk if you:
- are female
- are between the ages of 40 and 50
- have a hereditary disease that affects the
- have another form of cancer that is aggressive
Children under age 5 are also at higher risk for this condition.
Keep in mind, ACC is a rare cancer. Just because you have one or more risk
factors does not mean that you will get ACC.
What Are the Symptoms of an Adrenal Cortical
The symptoms of a functioning tumor depend on which hormones it
Testosterone and other androgens:
- increased facial and body hair, particularly in
- 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 ACC can cause Cushing’s, most of the tumors related to
the condition are benign. If you have Cushing’s, it does not mean you have
Diagnosing an Adrenal Cortical Carcinoma
To diagnose ACC, 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.
Treating an 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 are small tumors (less than 5 centimeters) that are still
within the tissues.
- Stage 2 tumors are large tumors (greater than 5 centimeters) that are still
within the tissues.
- Stage 3 tumors are tumors of any size that have spread to nearby lymph
nodes and fatty tissue.
- Stage 4 tumors are tumors of any size that have spread to other organs and
Depending on the stage of your cancer, a variety of treatments
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.
Prognosis: What Is to Be Expected in the
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
Your doctor will also monitor your health for other potential
problems related to ACC. 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.