Adrenocortical Carcinoma

What are the signs and symptoms of ACC?

The signs and symptoms of ACC in children are usually linked to an increase in hormones. These signs and symptoms can include high blood pressure, high blood sugar and excess weight gain. Early puberty is also often a sign, which results in increased facial and body hair and early breast development. A child with ACC can also form a lump or mass in the abdomen, which can cause abdominal pain.

What tests are used to diagnose ACC?

If your child has signs and symptoms of ACC, we’ll use an imaging test such as an MRI or a CT scan to examine their adrenal gland. Your child’s doctor may also recommend blood and urine tests to evaluate hormone levels. If a young child shows signs of early puberty, we usually perform blood and urine tests first to determine their hormone levels. Because these tumors can be related to genetic diseases like Beckwith-Wiedemann syndrome, your child’s doctor may also recommend genetic testing.

What to expect from an MRI or CT scan

An MRI is magnetic resonance imaging. We often use this device for imaging because there is no radiation involved. Your child will lie still for the test and it will not be painful. We do have distraction methods we can use to help your child lie still, including calming light displays. Your child may or may not need anesthesia for this test, depending on their age and level of mental development. The test takes about one hour.

A CT scan does have a small dose of radiation, but we calibrate our machines to use the least amount possible. Your child will also need to lie still for this test. This is a very quick scan that only takes a few minutes.

How do we make a diagnosis for ACC?

If a mass is found during the imaging tests, we may need to perform a biopsy. A biopsy is a procedure that is done to remove a small piece of tissue. We then examine the tissue under a microscope.

What to expect during a biopsy

We can perform a biopsy as an outpatient procedure. If your child is scheduled for a biopsy, we ask that they come with an empty stomach. We’ll place an IV in order to give them medication during the procedure and depending on the child, we’ll either sedate them or use full anesthesia to put them to sleep.

For the procedure, we insert a needle through the back and onto the surface of the adrenal gland. Using the needle, we’ll remove a few small pieces of tissue for examination. This part of the biopsy takes about one hour. We’ll monitor your child for several hours afterward to ensure there are no complications and then they will be allowed to go home.

Our team analyzes the tissue sample under a microscope and performs several tests, which take a few days to complete. Once the biopsy results are available, we will discuss them with you and your child and develop a personalized treatment plan together.

How is ACC treated?

ACC is typically treated using surgery to remove the tumor and the affected adrenal gland. ACC can often spread to other areas of the body, such as the lungs, before it has been diagnosed. If this is the case, we may also suggest a combination of chemotherapy or radiation treatment. This depends on the type, stage and location of the cancer.

Why choose us for treatment of ACC?

We have a dedicated surgical oncology team that specializes specifically in the surgical removal of tumors like the ones associated with ACC. Our surgeons will work closely with a multidisciplinary team to provide the most optimal care for your child. This team includes many specialists, including oncologists, pediatric surgeons, nephrologists, urologists, radiologists and pathologists.

In addition, our oncologic geneticists will provide consultation for long-term management and family considerations. Because these tumors are sometimes linked to genetic conditions, we may also recommend genetic screening for other family members.

Helpful resources

AdrenalCancerSupport.org provides links to online resources that focus on research and support for those with ACC and their families.