Adrenal Cortical Cancer

What Is Adrenal Cancer

The adrenals are small glands that sit above each of the kidneys. The kidneys are found deep inside the upper part of the abdomen.

The adrenal gland is made up of 2 parts. The outer part, called the cortex, is where adrenal cortical tumors start. The job of the cortex is to make certain hormones for the body. These hormones are called steroids.

The inner portion of the adrenal gland, called the medulla, is really a part of the nervous system. Nervous system hormones are made in the medulla. Tumors of the adrenal medulla are not covered in this article. The information here refers only to tumors of the adrenal cortex.

There are two main types of adrenal cortex tumors.

• benign (not cancerous) tumors, called adenomas
• adrenal cortical carcinoma; these tumors are cancerous

Most tumors of the adrenal cortex are benign adenomas. These are small tumors, usually less than 2 inches (5 centimeters) in diameter. They usually occur in only one adrenal gland, but sometimes are in both glands.

Most people with adenomas have no symptoms. They don�t know they have a tumor unless it is found when tests are being done because of other health problems. Some of these tumors make excess hormones, which may or may not cause symptoms. These symptoms are discussed in the section, "Can Adrenal Cortical Cancer Be Found Early?"

Adenomas can be cured by taking out the adrenal gland that contains the tumor. This operation can be done through a cut (incision) through the front of the belly (abdomen) or the back. It might also be done with laparoscopic surgery. To do this a thin tube with a tiny camera is inserted through a small cut in the patient�s side. Tools used through this small tube allow the surgeon to remove the adrenal gland with the tumor. Some adrenal adenomas can be treated with medicines. This might be a good option for patients with other medical problems who might not be able to withstand surgery. Still other small adenomas that are not causing any symptoms do not need any treatment at all.

The remainder of this document refers to adrenal cancers only, and not to adenomas.

Adrenal cortical carcinomas are usually found because of the symptoms they cause. They may make hormones that cause body changes such as weight gain, fluid build-up, early puberty in children, or excess facial or body hair in women. Some adrenal cancers get very large and cause symptoms by pressing on other organs. This can cause pain, a feeling of fullness, and weight loss because it is hard to eat very much. In most cases, adrenal cortical cancers are much larger than adenomas. If a tumor is larger than about 2 to 2-1/2 inches (5 or 6 centimeters), it is most likely cancer.

Some cancers start in other organs and then spread to the adrenal glands. For example, lung and breast cancers often spread to the adrenals. But these cancers are still named after the place where they started. They are not called adrenal cancers.

What Causes Adrenal Cancer?

A risk factor is anything that increases a person's chance of getting a disease. Different cancers have different risk factors. Some risk factors, such as smoking, can be controlled. Others, like a person's age or race, can't be changed. But scientists have found few risk factors that make a person more likely to get adrenal cancer. Even if a person does have one or more risk factors for adrenal gland tumors, we can�t know for sure how large a part they played in causing the cancer.

A family history: Most adrenal cancers are not linked to heredity. But a rare condition called Li-Fraumeni syndrome causes a higher risk of several types of cancer, including adrenal cancer.

Smoking has been suggested as a risk factor by some researchers.

How Is Adrenal Cancer Found?

It is hard to find adrenal cancer early. Often the tumor has grown quite large before it is found. It is often found earlier in children than in adults because children react more to the hormones these tumors make.

The symptoms of adrenal cancer can be caused by either the hormones they produce or because the tumor is pressing on nearby organs. If you or your child has any of the symptoms below, talk to your doctor right away. Getting the right tests is the only way to find out for sure what is causing the symptoms.

Signs and Symptoms of Adrenal Cancer

Symptoms caused by the hormones androgen or estrogen: In children, symptoms are most often caused by male-type hormones (androgens) that the tumor makes. These symptoms include:

• excess growth of facial, pubic, and underarm hair
• enlargement of the penis (boys)
• enlargement of the clitoris (girls)

A different set of symptoms can occur if the tumor makes female-type hormones (estrogens). These include:

• early puberty in girls (having periods and the breasts getting larger)
• breasts getting larger (boys)

In adults, the symptoms from sex hormones are harder to spot because these changes have already taken place. Women with tumors that make estrogen often do not have any symptoms unless the tumor is large enough to press on nearby organs. Men with tumors that make estrogen may have slight breast tenderness and enlargement. They may also notice less sex drive and trouble getting an erection.

Women with tumors that make androgens may notice the growth of excess facial and body hair, irregular periods, and deepening of their voice.

There is a group of symptoms called Cushing syndrome that results from high levels of a hormone called cortisol (or hormones like it). Some of the symptoms of Cushing syndrome include:

• weight gain, often around the chest and stomach
• fat deposits behind the neck and shoulders
• purple stretch marks on the stomach
• hair growth on the face, chest, and back in women
• irregular periods
• weakness in the legs
• easy bruising
• depression or moodiness
• broken bones from osteoporosis (thinning of the bones)

But there are several reasons for having high cortisol levels. Your doctor will need to do a number of tests to find out if the symptoms are from adrenal cancer or some other cause.

If the tumor makes a hormone called aldosterone, the main symptoms include:

• high blood pressure
• weakness
• muscle cramps
• increased thirst
• urinating very often

Most often, though, the aldosterone is made by an adenoma rather than by cancer.

If the tumor is large and presses on nearby organs and tissues it can cause pain, a feeling of fullness in the stomach, or trouble eating because the stomach feels full.

If you have any of the signs or symptoms above, talk to your doctor right away. Remember, the sooner your cancer is found, the sooner you can start treatment. And the earlier you get treatment, the more effective it will be.

If there is any reason to suspect cancer, the first step will be a complete medical history and physical exam. Your doctor will want to know if anyone in your family has had adrenal cancer. You�ll also be asked about your periods or other sexual issues and what symptoms you have had. One or more of the tests below may also be done.

Imaging Tests

Chest x-ray: This can tell if the cancer has spread to the lungs. It may also help to see if you have any lung or heart diseases.

Ultrasound: This test uses sound waves to make pictures of internal organs. The computer displays the picture on a screen. This test will be able to show if there is a tumor mass in the adrenal gland. It can also show if there is a tumor in the liver. Most likely it won�t be used unless a CT scan can�t be done.

CT scans (computed tomography): A CT scan uses x-rays to make detailed pictures of your body. Instead of taking just 1 x-ray, a CT scanner takes many pictures as it moves around you. A computer then combines these into a picture of a slice of your body. CT scans can show the adrenal glands and can often confirm whether a tumor is present, how large it is, and whether it has spread to nearby sites. CT scans also show the organs near the adrenal glands, as well as lymph nodes and distant organs. This test can help show if the cancer has spread to the liver or other organs. It also may be used to guide a needle into an area of concern. Once the needle is in, a piece of tissue is removed to be looked at under a microscope. This is called a CT-guided needle biopsy.

CT scans take longer than regular x-rays and you need to lie still on a table while they are being done. Also, you might feel a bit confined by the machine you lie in while the scan is being done. But CT scans are getting faster and your stay might be pleasantly short.

PET scan (positron emission tomography): For a PET scan, a type of radioactive sugar is injected into a vein. The sugar collects in cancer cells. A scanner can spot these deposits. This test is useful for finding cancer that has spread beyond the adrenal glands. It also helps to stage the cancer. Some doctors feel it can be useful in telling whether a tumor in the adrenal gland is cancer or not.

MRI (magnetic resonance imaging): MRI scans use radio waves and strong magnets instead of x-rays. MRI pictures look a lot like those of a CT scan, but MRIs are more detailed. An MRI scan can also show views from several angles. The MRI may sometimes provide more information than a CT scan because it can better show the difference between adrenal cancer and a benign tumor. An MRI is especially helpful in looking at the brain and spinal cord.

MRI scans can be a little more uncomfortable than CT scans because they take longer, often up to an hour. Also, you must lie still inside a tube, which may be upsetting to some people. The machine makes a thumping noise as the magnet switches on and off. Some places have earplugs or headphones with music to block this out.

Other Tests

Laparoscopy (lap-uh-ros-kuh-pee): The doctor may do this test in order to get ready for surgery. The laparoscope is a thin, flexible tube with a tiny video camera on the end. It is inserted through a small opening in the patient�s sideto look at the tumor. It can be used to make sure all the cancer can be removed.

Biopsy: In a biopsy a sample of tissue is removed to see whether cancer cells are present. This test may be done before surgery by using a needle that removes small pieces of tissue. A CT scan or ultrasound might be used to help guide the needle. The results can show whether the tumor started in the adrenal cortex, the medulla, or some other part of the body. But it might not show whether the tumor is cancer or not. For this reason, surgery is done if the tumor�s size and certain features suggest it is cancer. If it looks as if the cancer has spread to another part of the body such as the liver, then a biopsy may be done there, too.

Blood and urine tests: These tests are important in deciding whether a patient with symptoms of adrenal cancer has the disease. Doctors choose which tests to do based on the patient�s symptoms. Because they know which symptoms are linked with high levels of certain hormones, they can select the right test for the patient.

How Is Adrenal Cancer Treated?

This information represents the views of the doctors and nurses serving on the American Cancer Society's Cancer Information Database Editorial Board. These views are based on their interpretation of studies published in medical journals, as well as their own professional experience.

The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your family make informed decisions, together with your doctor.

Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. Don't hesitate to ask him or her questions about your treatment options.

After the cancer is diagnosed and staged, your doctor will talk to you about your treatment choices. Take time and think about all of the choices. In choosing a treatment plan, factors to think about include your overall health and the stage of the cancer. You might also want to check with another doctor for a second opinion. This can give you more information and help you feel good about the choice you make. Another reason to look for a second opinion is that these cancers are rare. Only large cancer centers will have much experience in treating them.

The main methods of treatment for cancer are surgery, radiation therapy, chemotherapy, and the use of other drugs.

Surgery

To remove most adrenal cortical tumors, the surgeon will make an incision through the front of the abdomen. If the cancer has grown into the kidney, it will be removed as well. If it has grown into the muscle and fat around the gland, these tissues will also be removed. If the liver is involved, part of that organ will be removed. If the cancer has grown into the large vein that carries blood from the lower part of the body to the heart (the inferior vena cava), an extensive operation will need to be done. The goal of surgery is to get out as much of the cancer as possible, no matter where it is found.

Radiation Therapy

This treatment uses high-energy radiation to kill cancer cells. But radiation therapy is not often used for adrenal cancer because the x-rays do not easily kill this type of cancer.

Chemotherapy

Chemotherapy (most often called chemo) refers to the use of drugs to kill cancer cells. Usually the drugs are given into a vein or by mouth. Once the drugs enter the bloodstream, they reach throughout the body. Chemo is not very good treatment for adrenal cancer. It is only used to treat adrenal cancer that has become widespread.

Chemo can have some side effects. These side effects will depend on the type of drugs given, the amount taken, and how long the treatment lasts. Side effects could include the following:

• nausea and vomiting
• loss of appetite
• hair loss (hair grows back after treatment)
• hand and foot rashes
• mouth sores
• increased chance of infection (from low white blood cell counts)
• bleeding or bruising easily (from a shortage of blood platelets)
• tiredness (from low red blood cell counts)

If you have side effects, your doctor or nurse can suggest steps to ease them. For example, there are drugs to help control and prevent nausea and vomiting. The good news is that most side effects will go away when your treatment ends.

Other Drugs to Treat Adrenal Cancer

A drug called mitotane is most often used to treat people with adrenal cancer. Mitotane blocks the gland from making hormones and also destroys cancer cells. Studies have shown that starting mitotane treatment when all of the cancer is thought to be gone may delay the return of the cancer. For some patients whose cancer was not completely removed by surgery or whose cancer has come back, this drug will shrink the cancer. Even when it doesn�t shrink the tumor, it can help relieve symptoms. About 8 out of 10 patients are helped by mitotane. On average, the response lasts about one year, or sometimes longer.

Unlike chemo drugs, mitotane does not cause low blood counts. But it can cause major side effects. The most common are nausea, vomiting, diarrhea, rashes and sleepiness. Sometimes lower doses of the drug can be used with fewer side effects. There are also some other drugs that may work for some people.

Adrenal Cancer Survival Rates

Because the number of cases of adrenal cancer is so small, the survival rates are only estimates. Also, the cancer itself has certain features that make it more or less likely to grow fast and spread. While the numbers below provide an overall picture, keep in mind that every person�s situation is unique and the statistics can�t predict exactly what will happen in your case. Talk with your cancer care team if you have questions about your personal chances of a cure, or how long you might survive your cancer. They know your situation best.

The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is found. Of course, many people live much longer than 5 years. These rates are also based on people first treated more than 5 years ago. Improved treatments often mean better survival rates for patients now.

Approximate Survival Rates for Adrenal Cortical Cancer

Stage	Survival Rate
I	50% to 60%
II	50% to 60%
III	20%
IV	Less than 10%