Adrenal Cancer

What is adrenal cancer?

Adrenal cancer occurs when cells in your adrenal glands mutate (change) and grow, forming a tumor. You have two adrenal glands — one on top of each kidney. They make hormones that send signals to many different organs to help keep your body healthy.

You can get adrenal cancer in one or both adrenal glands.

Types of adrenal gland cancer

There are three main types of adrenal cancer. Each starts in a different area of your adrenal gland:

1. Adrenocortical carcinoma (ACC). This is the most common type of adrenal cancer. It forms in the outer layer of your adrenal gland (the cortex). Your cortex makes steroid hormones that control elements like metabolism, blood pressure, body shape and hair growth. Another name for adrenocortical carcinoma is adrenal cortex cancer.
2. Neuroblastoma. Neuroblastomas form in nerve cells, so they can affect many different parts of your body. However, about one-third form in the inner part of your adrenal gland (the medulla). The medulla makes epinephrine and norepinephrine. These chemicals help control your sympathetic nervous system, which regulates things like sweating, heart rate and blood pressure. Adrenal neuroblastomas usually affect infants and children under the age of 10.
3. Pheochromocytoma. This adrenal gland cancer starts in the center of your medulla, usually in the cells that produce adrenaline. Adrenaline helps regulate your blood pressure and heart rate.

How common is adrenal gland cancer?

Adrenal gland cancer is rare, affecting about 200 people in the United States every year.

What are the symptoms of adrenal cancer?

Adrenal cancer affects everyone differently. Some people develop pain if the tumor grows and presses on nearby organs. Others may notice symptoms based on the extra hormones the tumor releases.

General adrenal cancer symptoms may include:

• Abdominal pain.
• Back pain.
• Feeling of fullness in your belly.
• Muscle cramps.
• Weakness.
• Headaches.

Hormone-specific adrenal cancer symptoms might include:

• Excess facial or body hair (hirsutism).
• Newly elevated blood sugar (diabetes).
• High blood pressure.
• Low potassium (hypokalemia).
• Unexplained weight loss.
• Unexplained weight gain.
• Fast heartbeat.
• Enlarged breasts.
• Enlarged penis or clitoris.
• Low sex drive (low libido).
• Anxiety.
• Panic attacks.

In some cases, adrenal gland tumors don’t cause any symptoms.

Metastatic adrenal cancer symptoms

People with metastatic adrenal cancer (cancer that spreads from your adrenal glands to other parts of your body) might develop more severe symptoms as the disease progresses. Possible Stage 4 adrenal cancer symptoms include:

• Nausea and vomiting.
• Bloating.
• Fatigue.
• Fever.
• Confusion.
• Loss of appetite.

What causes adrenal cancer?

Healthcare providers don’t know what causes most adrenal gland cancers. They know that mutations in the DNA can cause cells in your adrenal gland to change, grow and become cancerous. But they don’t know exactly why these DNA mutations happen.

Some people inherit a gene mutation from a biological parent, which increases their risk of adrenal cancer. Others may develop adrenal gland cancer due to exposure to certain cancer-causing substances. But in the majority of cases, these DNA mutations seem to happen for no apparent reason.

Adrenal cancer risk factors

A risk factor is something that increases your chance of developing a certain cancer or disease. It doesn’t mean that you’ll get the disease. But it helps you know you’re at risk.

Possible environmental risk factors for adrenal cancer include:

• Smoking.
• Exposure to certain chemicals like asbestos and radon.
• Prior radiation therapy to your abdomen, where your adrenal glands are located.

While most adrenal cancers occur for no apparent reason, about 15% of people who develop adrenal tumors have a genetic disorder. This is most common in children. Genetic disorders associated with adrenal gland cancer include:

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

How is adrenal cancer diagnosed?

Your healthcare provider may suspect cancer in your adrenal glands based on your specific symptoms, such as excessive hair growth or unexplained weight changes. But sometimes, providers find adrenal tumors after taking a CT (computed tomography) scan or MRI (magnetic resonance imaging) for other reasons.

During a visit with your healthcare provider, they’ll:

• Perform a physical examination.
• Review your medical history.
• Ask about your symptoms.
• See if you have any known risk factors for adrenal cancer.
• Review your family history of cancer.

What tests can help diagnose adrenal cancer?

After your exam, your healthcare provider will run additional tests to confirm your diagnosis or rule out other conditions. These tests may include:

• Blood tests.
• Urinalysis (urine tests).
• Imaging tests.

Adrenal cancer staging

Healthcare providers use a cancer staging system to diagnose adrenal cancer. Staging tells you the size and location of the tumor and whether it has metastasized (spread to other areas of your body).

In general, the higher the number, the more advanced the tumor:

• Stage 1: The tumor measures 5 centimeters or less and hasn’t spread outside of your adrenal gland.
• Stage 2: The tumor measures more than 5 centimeters and hasn’t spread outside of your adrenal gland.
• Stage 3: The cancer is in your adrenal gland and has spread to surrounding tissues or nearby lymph nodes.
• Stage 4: The cancer is in your adrenal gland and has spread to distant areas of your body, such as your lungs or liver.

Staging systems can be complex. If you have specific questions about your diagnosis, you should talk to your healthcare provider.

Is adrenal cancer curable?

There’s a chance for a cure when the tumor is only in your adrenal gland and hasn’t spread to other areas of your body. Surgical removal (adrenalectomy) is the main curative treatment for adrenal cancer.

If the cancer has spread beyond your adrenal gland, a cure becomes less likely. But treatment slows the growth of the tumor and improves your quality of life.

How is adrenal cancer treated?

Adrenal cancer treatment depends on the size and location of the tumor, and whether it has spread outside of your adrenal gland. Possible treatments include:

• Surgery.
• Radiation therapy.
• Chemotherapy.
• Other medications.

Surgery

If the tumor is in your adrenal gland or has only spread to very nearby tissues or lymph nodes, then your provider will likely recommend surgery. A surgeon will remove your entire adrenal gland (adrenalectomy) and any diseased structures next to it.

If cancer is only in one adrenal gland, then your other adrenal gland should work properly. In many cases, you don’t need to do anything else. If you need to have both adrenal glands removed, then you’ll need to take medicine for the rest of your life to replace the hormones your glands produce.

Sometimes, providers recommend chemotherapy or radiation therapy after surgery to kill any remaining cancer cells and reduce the risk of recurrence (return).

In some cases, providers may recommend surgery even after the cancer has spread to remove as much of the tumor as possible. Providers call this debulking surgery.

Radiation therapy

Radiation therapy uses high-energy X-ray beams to kill cancer cells. Providers usually don’t use radiation therapy as the first treatment for adrenal cancer. They might use radiation therapy after surgery to decrease the chance of cancer coming back. If adrenal cancer has spread, they might use radiation therapy to treat sites of spread to reduce symptoms. Healthcare providers may combine radiation with chemotherapy or other medicines.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. Your provider may give chemotherapy drugs in pill form or through a vein (intravenously). Providers often use chemotherapy to treat adrenal cancer that has spread to other areas of your body. Or they may use it following surgery to kill any remaining cancer cells.

Mitotane is the most common chemotherapy drug used to treat cancer in your adrenal glands.

Other medications

Healthcare providers may use other medications to treat adrenal gland cancer. Some drugs, like metyrapone, can reduce adrenal steroid hormone production. Other drugs, like spironolactone and mifepristone, block the effects of the hormones that the tumor releases.

These drugs can ease your symptoms, but they don’t shrink or kill cancer cells.

Who treats adrenal cancer?

If you have adrenal cancer, your medical team may include several specialists, including:

• Oncologists (surgical, radiation and medical).
• Endocrinologists.

Can I prevent adrenal cancer?

There’s no proven way to prevent adrenal gland cancer. Avoiding environmental risk factors, like smoking, may help reduce your overall risk.

Does adrenal cancer spread fast?

It depends on the type of tumor you have:

• Adrenocortical carcinoma, the most common type of adrenal cancer, usually grows rapidly and spreads quickly.
• Some neuroblastomas grow slowly, but others grow quickly.
• Most cancerous pheochromocytomas grow slowly.

Adrenal cancer affects everyone differently. If you have questions about how fast an adrenal tumor may grow, talk to your healthcare provider.

How serious is cancer in the adrenal gland?

In general, adrenal gland cancer is difficult to cure. Adrenal cancer prognosis depends on the location and size of the tumor and whether it has spread to other areas of your body.

Can you survive an adrenal tumor?

Yes, it’s possible to survive an adrenal gland tumor. The outlook for people with adrenal gland cancer varies significantly depending on the location of the tumor.

Adrenal cancer survival rates

The five-year survival rate for early-stage adrenal cancer is between 50% and 60%. This means that up to 60% of people with this disease will still be alive five years after their diagnosis. Once the cancer spreads to other areas of your body, the five-year survival rate drops to 10% to 20%.

Survival rates can’t tell you how long you’ll live or how well a certain treatment will work for you. To learn more about survival rates and what they mean in your specific situation, talk to your healthcare provider.

When should I see my healthcare provider?

It’s not always possible to spot the symptoms of adrenal cancer. But you should schedule an appointment with your healthcare provider if you develop:

• Abdominal pain.
• Sudden-onset diabetes.
• Unexplained weight loss or weight gain.
• Excessive hair growth.
• Changes to your genitals.

If a first-degree biological relative (parent or sibling) develops adrenal cancer, consider talking to your provider about your risk.

What questions should I ask my healthcare provider?

If you or a loved one have an adrenal cancer diagnosis, you may want to ask these questions:

• Where is the tumor?
• What size is the tumor?
• Has the cancer spread outside of my adrenal gland?
• Do I need more testing?
• What are my treatment options?
• Will I be able to work or go to school during treatment?
• When will I start treatment?
• What can I do to ease my symptoms?
• What are the chances that adrenal cancer will come back?

A note from Cleveland Clinic

Hearing that you have adrenal cancer can bring up a lot of complicated emotions. Your healthcare provider is here to help. In addition to helping you explore treatment options, they can recommend resources to inform and support you. Local or online groups offer a way for you to talk with others who are going through the same thing. Resources like these can benefit you mentally, emotionally and spiritually as you navigate this difficult time.