Adrenocortical Carcinoma

What is adrenocortical carcinoma?

Adrenocortical carcinoma is cancer in your adrenal cortex, the outer layer of your adrenal glands. These glands are on top of your kidneys and produce hormones that, among other things, manage your body’s metabolism, blood pressure and how you react to stress.

In adrenocortical carcinoma, there are cancerous tumors on your adrenal glands that may release abnormally large amounts of hormones. The flood of hormones may affect how your body works. Fast-growing tumors may press on nearby organs. In some instances, healthcare providers can treat adrenocortical carcinoma, but the condition often comes back (recurs).

This condition typically affects adults, but children sometimes develop it, too. This article focuses on adrenocortical carcinoma in adults.

What are types of adrenocortical carcinoma?

There are two types — functioning and non-functioning — that cause different symptoms:

• Functioning tumors: Most adrenocortical carcinomas are functioning. They release excessive amounts of hormones, including aldosterone, cortisol, estrogen and testosterone. Symptoms vary depending on the type of hormone.
• Non-functioning tumors: These tumors don’t affect hormone production, but they can grow so large that they press on nearby organs and tissues.

How common is this condition?

Adrenocortical carcinoma is very rare even, though it’s the most common type of adrenal cancer. Each year, about 1 in 1 million people receive a diagnosis of adrenocortical carcinoma.

What are the symptoms of adrenocortical carcinoma?

You can have this condition without having symptoms. Studies show that 20% to 30% of adrenocortical carcinoma diagnoses happen after healthcare providers do imaging tests for unrelated health issues. When adrenocortical carcinoma symptoms develop, they may include:

• Abdominal (belly) pain.
• Hirsutism (excess body or facial hair in women and people assigned female at birth).
• Gynecomastia (breast enlargement) in men and people assigned male at birth.
• High blood pressure.
• High blood sugar.
• Weight gain in your face, neck and trunk.

How aggressive is adrenocortical carcinoma?

It’s very aggressive because the tumors can grow very quickly and spread (metastasize) from your adrenal gland to other areas of your body, including your lungs or bones.

What causes adrenocortical carcinoma?

Researchers don’t know the exact cause. Some people develop adrenocortical carcinoma because they have inherited conditions that increase the risk that they’ll develop the condition.

In other cases, certain genetic mutations (changes) appear to increase risk. For example, research shows changes in the tumor suppressor genes TP53 and IGF2 appear to drive adrenocortical carcinoma. Tumor suppressor genes manage cell growth. When these genes change, your cells may multiply uncontrollably and become cancerous tumors.

What inherited conditions increase my risk?

Having any of the following conditions increases your risk of developing adrenocortical carcinoma:

• Beckwith-Wiedemann syndrome.
• Carney complex.
• Familial adenomatous polyposis (FAP).
• Li-Fraumeni syndrome.
• Lynch syndrome.
• Multiple endocrine neoplasia (MEN1).
• Neurofibromatosis Type 1 (NF1).
• Von Hippel-Lindau (VHL) syndrome.

What are the complications of adrenocortical carcinoma?

It can spread (metastasize) very quickly. Cancer that spreads from your adrenal gland to other areas of your body is more difficult to treat. Tumors that release certain hormones may cause conditions including:

• Cushing syndrome: This condition happens when adrenal glands release too much cortisol.
• Conn’s syndrome: Excess aldosterone causes this condition.

How is adrenocortical carcinoma diagnosed?

If tests for other issues reveal a tumor on your adrenal gland or you have certain symptoms, your healthcare provider will do the following:

• Imaging tests, such as MRI, CT scan or PET scan to look for tumors.
• Blood tests and urinalysis to check hormone levels.

Your provider may do a biopsy to confirm that a tumor is cancerous, as well as obtain tissue samples.

What are stages of adrenocortical carcinoma?

Healthcare providers use cancer staging systems to plan treatment and develop a prognosis (what you can expect to happen after treatment). They establish adrenocortical carcinoma stages by evaluating tumor size and location and whether the tumor has spread to nearby lymph nodes or other more distant organs. Adrenocortical carcinoma stages include:

• Stage I: The tumor is 5 centimeters (about 2 inches) or less and hasn’t spread outside your adrenal gland.
• Stage II: The tumor is larger than 5 cm but hasn’t spread outside your adrenal gland.
• Stage III: The tumor has spread to nearby lymph nodes.
• Stage IV: The tumor has spread to nearby lymph nodes, nearby organs or more distant organs.

Cancer staging information can be confusing, so don’t hesitate to ask your healthcare provider to explain the system and how it applies to your situation.

What are treatments for adrenocortical carcinoma?

The most common treatment is adrenalectomy to remove one or both of your adrenal glands. Often, healthcare providers prescribe additional medications like:

• Mitotane (Lysodren^®): This drug limits adrenal gland activity, which may reduce the risk that adrenocortical carcinoma will come back. Providers use this medication after surgery to remove one adrenal gland or if surgery isn’t an option. Because this medication shuts down hormone production, people who receive it also take replacement medication.
• Metyrapone (Metopirone^®): Providers may prescribe this and similar drugs to ease symptoms that happen when tumors produce excess hormones.

In some cases, however, the condition isn’t diagnosed until after tumors have grown too large to be safely removed with surgery. In those instances, healthcare providers may treat cancerous tumors with chemotherapy. This treatment won’t eliminate the cancerous tumors, but it can ease symptoms and slow down tumor growth.

You also may want to consider palliative care as part of your treatment. Palliative care is treatment that helps manage disease symptoms and treatment side effects. Palliative care specialists can also help you with emotional and mental health support.

What are complications or side effects of treatment?

Surgery and medications may cause different complications or side effects. For example, surgery to remove an adrenal gland may:

• Affect hormone production: Usually, your remaining adrenal gland takes over for the missing gland to make enough hormones (adrenal insufficiency). When that doesn’t happen, you may need to take replacement hormone tablets until your remaining gland starts to produce enough hormones.
• Cause infection: You may develop an infection in your abdomen or the surgical site.
• Cause excessive bleeding: This can happen during or after surgery.

What are medication complications or side effects?

Your healthcare provider may recommend that you take mitotane for two to five years after surgery. Mitotane side effects may include:

• Nausea and vomiting.
• Diarrhea.
• Rashes.
• Confusion.

Can adrenocortical carcinoma be prevented?

No, it can’t. Researchers know about half of all cases of adrenocortical carcinoma happen when certain genes mutate and create cancerous cells that multiply and become tumors. But they don’t know what triggers those mutations, so they can’t recommend ways you could prevent them from happening.

But some inherited disorders increase your risk of developing this condition. If you have a family history of one of these disorders, talk to a healthcare provider about genetic tests that detect the mutations that cause these disorders.

What are survival rates for adrenocortical carcinoma?

Overall, 50% of people with adrenocortical carcinoma were alive five years after diagnosis. Survival rates vary widely, depending on factors like:

• Tumor stage at diagnosis.
• Whether the tumor was functioning (producing hormones) or nonfunctioning.
• Your age.
• Your overall health.

Five-year survival rates by stage are:

Survival rate information may make you feel anxious or afraid. That’s understandable. Try to remember that these estimates are based on experiences that happened in the past. Experts measure survival rates every five years. Many things can change in five years that may make a difference. It’s also important to remember that survival rates aren’t estimates for how long you will live.

If you have specific questions about cancer survival rates, talk to your healthcare provider. They’re your best resource because they know your situation.

How do I take care of myself?

Adrenocortical carcinoma is a rare cancer that often comes back — two challenges that may make you feel afraid, isolated or anxious. Fortunately, there are steps you can take that may help:

• Find support: Ask your healthcare team about support groups for people with adrenocortical carcinoma. It may help to spend time with or talk to people who know what you’re going through.
• Consider cancer survivorship programs: Many people with adrenocortical carcinoma feel anxious about cancer coming back. Cancer survivorship programs are one resource for dealing with that anxiety.
• Continue palliative care: You may need post-surgery chemotherapy and want help managing treatment side effects.

When should I see my healthcare provider?

Adrenocortical carcinoma often comes back (recurs), most commonly within the first two years after surgery Your provider will set up a schedule of follow-up appointments to monitor for signs of recurrence. For example, you may have:

• Imaging tests: You may have tests every three months for two years, and then every three to six months for three more years.
• Blood tests: Your provider will do regular tests to check on hormone levels.

What questions should I ask my healthcare provider?

Adrenocortical carcinoma is a rare disease. You may have many questions about what you can expect. Here are some questions you may find helpful:

• What does it mean if a tumor is functioning or non-functioning?
• Has the tumor spread outside my adrenal gland?
• If so, how far has it spread?
• What are my treatment choices?
• What are treatment side effects?
• Should my family members have genetic testing for the mutations that cause adrenocortical carcinoma?
• Assuming treatment works, what are the chances the tumor will come back?