What Is Adrenocortical Carcinoma?

Adrenocortical carcinoma, or cancer of the adrenal cortex, is a rare form of cancer that affects the outer layer of the adrenal glands. These glands are located on top of both kidneys and produce hormones that regulate important body processes.

This type of cancer occurs in less than one person per million per year and in more females than males (male-to-female ratio is 1:1.5). It is mostly diagnosed in people in their forties and fifties, but can occur in children (most often with Li-Fraumeni syndrome).

While adrenocortical carcinoma is very rare, it’s one of the most common adrenal gland cancers.

Adrenocortical Carcinoma Symptoms

The adrenal cortex is responsible for producing hormones that help regulate bodily functions. In some cases, adrenocortical carcinoma can affect hormone production, causing symptoms. This occurs when an adrenal cortex tumor is functioning. A non-functioning tumor won’t cause hormone imbalances.

Patients may present either with:

• Symptoms due to overproduction of hormones (40%)
• Abdominal pain or fullness or flank pain (40%)
• No symptoms, but the tumor is found during an unrelated workup (20%)

Symptoms due to hormone secretion depend on the hormones secreted. Most hormonally active tumors secrete androgens, cortisol, or a combination of both.

Symptoms from excess hormones can include:

• Glucocorticoids such as cortisol: Causing symptoms of Cushing’s syndrome such as weight gain, diabetes, muscle weakness
• Androgens such as testosterone: Causing no symptoms in males, but in females increasing body hair growth, balding, acne, and changes in the menstrual cycle
• Estrogens (rare): Growth of breast tissue in males, menstrual irregularities in females
• Mineralocorticoids such as aldosterone: Causing high blood pressure and low potassium levels
  

Symptoms of hormone overproduction are more likely to cause noticeable symptoms in children than in adults.

When adrenal cortex tumors don’t produce excess hormones, they may not cause symptoms at all. A person may only notice symptoms as the cancer gets larger or spreads. 

Causes

Experts don’t fully know what causes adrenocortical carcinoma. However, certain DNA mutations or genetic syndromes may increase a person’s risk of developing this type of cancer.

Genetic conditions that may play a role in the development of adrenal cortex tumors include:

• Li-Fraumeni syndrome
• Beckwith-Wiedemann syndrome
• Familial adenomatous polyposis (FAP)
• Lynch syndrome
• Multiple endocrine neoplasias (MEN1)
• Von Hippel-Lindau syndrome 
• Carney complex

Diagnosis

People with adrenocortical carcinoma often find out they have cancer while getting tests or imaging done for other conditions. For example, if a doctor suspects a person has Cushing’s syndrome, abnormal hormone level results may lead to further testing.

Exams and procedures used to diagnose adrenocortical carcinoma include:

• Physical exam: The doctor will ask about your symptoms. They may look for obvious signs of hormone imbalance, such as having a round, full face.
• Medical and family history: The doctor will ask whether you have a family history of cancer or a hereditary condition that may increase your risk of developing this type of cancer.
• Urine test to check for cortisol or 17-ketosteroid levels: High levels of either may be a sign that there’s a problem with the adrenal cortex.
• Dexamethasone suppression tests: These tests can check whether the adrenal gland is overproducing cortisol. They can also check whether the pituitary gland is the cause of hormone overproduction.
• Blood chemistry study: Abnormal blood levels of certain substances may indicate a problem.
• Biopsy: During this procedure, a healthcare professional takes a sample of tissue to examine and test. This may happen if a doctor notices a growth during imaging. 
• Computed tomography (CT) scan: This imaging test allows the doctor to check your internal organs and tissues.
• Magnetic resonance imaging (MRI): An abdominal MRI may help diagnose adrenocortical carcinoma. 
• Positron emission tomography (PET) scan: This type of imaging checks for the presence of cancer cells. 
• Adrenal angiography: This procedure allows the doctor to check for blockage of the adrenal arteries.
• Adrenal venography: This procedure allows the doctor to check for blockage in the adrenal veins.
• MIBG scan: This nuclear imaging study may be done to check for the presence of pheochromocytoma, another type of adrenal gland tumor. It uses a small amount of radioactive MIBG (iodine-131-metaiodobenzylguanidine) that gets taken up by cells such as seen in pheochromocytoma.

None of these tools can diagnose adrenocortical carcinoma alone. A doctor can use them in combination to make a diagnosis. 

When diagnosing adrenocortical carcinoma, a doctor will also stage the cancer according to its size and whether it has spread to other areas.

Treatment

The only chemotherapy agent approved for the treatment of adrenocortical carcinoma is mitotane.

Treatment for adrenocortical carcinoma depends on the cancer’s staging. In the early stages, the first-line treatment is typically the removal of the affected adrenal gland, also called adrenalectomy.

Doctors may also recommend chemotherapy or radiation post-surgery. This is called adjuvant therapy. It aims to kill any remaining cancer cells and minimize the risk of cancer returning.

In the later stages, when cancer has spread elsewhere, doctors may recommend mitotane alone or in combination with other chemotherapy agents.

Doctors may also recommend surgery and radiation as palliative care measures when the cancer is in the later stages. These procedures can improve a person’s quality of life by offering symptom relief, but they are not expected to be curative.

Biologic and targeted therapies are currently being researched as possible treatments for adrenocortical carcinoma. Biologic therapy or immunotherapy uses the body’s immune system to attack the cancer. Targeted therapy drugs attack specific cancer cells without harming healthy ones.

So far, neither targeted agents nor immunotherapy has proven their efficacy in this condition, and clinical research studies are ongoing.

Prognosis

The prognosis for people with adrenocortical carcinoma depends on the stage of the cancer. The estimated five-year survival rates for people with adrenal cancer are:

• Localized: 74%
• Regional: 56%
• Distant: 37%
  

Coping

Getting a diagnosis of adrenocortical carcinoma can be devastating. Even if caught in the early stages, hearing the word “cancer” can feel overwhelming.

Here are a few suggestions for coping with your cancer diagnosis:

• Seek out support: Ask family and friends for help. Be specific with your requests. Do you need rides to appointments? Are you struggling to cook for yourself and need a helping hand? Additionally, cancer support groups can offer another support system. They can help to connect you to people who know exactly what you’re going through.
• Talk to your doctor: Are you dealing with side effects? Ask your doctor about options for managing side effects. 
• Look into financial assistance: Cancer treatment is expensive. Reduce financial stress by looking into financial support for people living with cancer. The Cancer Financial Assistance Coalition, for example, can offer you financial resources and guidance.

Summary

Adrenocortical carcinoma is a rare form of cancer that begins in the outer layer of the adrenal glands. If the tumor produces hormones, it can cause symptoms related to hormone imbalance. If it doesn’t, there may not be symptoms unless it spreads.

This rare cancer doesn’t have known causes other than being associated with certain genetic conditions. It is diagnosed with imaging, blood and urine tests, and a biopsy. Treatment in early stages is by surgically removing the adrenal gland. In more advanced stages it may be treated by surgery, chemotherapy, or radiation therapy.

A Word From Verywell

“You have cancer.” When a doctor uses these words, it may feel like your life is falling apart. Getting a cancer diagnosis can be scary and overwhelming. But there are treatment options available, even for this rare form of cancer. 

Getting an early diagnosis means you’re more likely to have a better outcome. If you notice weird symptoms that might signal that you have a hormone imbalance, don’t ignore them. Talk to your doctor if you have a family history of genetic diseases associated with adrenal cortex cancer. This alerts them to keep a close eye on your adrenal gland health.