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 two parts:
- The cortex (the outer part) is where adrenal cortical tumors start. The job of the cortex is to make certain hormones, called steroids, for the body.
- The medulla ( the inner portion of the adrenal gland) is really a part of the nervous system. Nervous system hormones are made in the medulla.
There are two main types of adrenal cortex tumors.
- Adenomas - benign (not cancerous) tumors
- Adrenal cortical carcinoma - these tumors are cancerous
Most people with adenomas have no symptoms. Many of these tumors do not make excess hormones, but some do. The excess hormones may or may not cause symptoms. Adenomas can be cured by taking out the adrenal gland that contains the tumor. This might be done with laparoscopic surgery. A thin tube with a tiny camera is inserted through a small opening in the patient’s side. This allows 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.
Adrenal cortical carcinomas, on the other hand, 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. In most cases, adrenal cortical cancers are much larger than adenomas. If a tumor is larger than about 2 to 2 1/2 inches, it is most likely cancer.
Some cancers start in other organs and then spread to the adrenal glands. For example, lung cancer often spreads to the adrenals. But these cancers are still named after the place where they started. They are not called adrenal cancers.
How Many People Get Adrenal Cancer?
- Adrenal cancer is very rare.
- The exact number found in the United States is not certain. It is probably around 300-500 per year.
- The average age of those found with adrenal cancer is around 45 but it can happen at any age.
- It seems to occur more often in women.
What Causes Adrenal Cancer?
Risk factors for Adrenal Cancer include:
- 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.
- Age: Most types of cancer are found more often in older people. But adrenal cancer is found most often among adults around ages 40 to 50 and among children.
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.
What are the signs and symptoms of Adrenal Cancer ?
In children, symptoms are most often caused by male-type hormones (androgens) that the tumor makes. These symptoms include:
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.
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 (benign) rather than by cancer.
If the tumor 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 images of internal organs. The computer displays the image 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 images of your body. Instead of taking just one x-ray, a CT scanner takes many pictures as it moves around you. A computer then combines these into an image of a slice of your body. CT scans can show the adrenal glands fairly clearly and can often confirm whether a tumor is present, how large it is, and whether it has spread to nearby sites. CT scans can 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. CT scans can also be used to guide a biopsy needle into a mass. Once the needle is in, it removes a sample of tissue to be looked at under a microscope. 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 cancerous tissue. A scanner can spot these deposits. This test, which is still being studied, is useful for finding cancer that has spread beyond the adrenal glands. It may be a very useful test for staging the cancer.
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 make you feel confined. The machine makes a thumping noise as the magnet switches on and off. You may be given earplugs or headphones with music to block this out.
Other Tests
Laparoscopy : 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 side. It may 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 usually won’t show whether the tumor is cancer or not. For this reason, surgery is done if the tumorÂ’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 there may be done.
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.
Staging
Staging is the process of finding out how far the cancer has spread. This is very important because the type of treatment chosen and the outlook for recovery depend on the stage of the cancer. Stages are expressed in Roman numerals I through IV (1-4). A higher number means a more serious cancer.
Stage I: The cancer is smaller than 2 inches. It has not spread to nearby tissues, organs, lymph nodes, or other places in the body.
Stage II: The cancer is larger than 2 inches but it has not spread to nearby tissues, organs, lymph nodes, or other places in the body.
Stage III: The cancer can be any size, but it has grown into nearby tissues or has spread to nearby lymph nodes, but not any farther.
Stage IV: The cancer can be any size and it has spread to distant places in the body.
How Is Adrenal Cancer Treated?
After the cancer is diagnosed and staged, your doctor will talk to you about your treatment choices. The main methods of treatment for adrenal 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.
- And if the cancer has grown into the large vein that carries blood from the lower part of the body to the heart, an extensive operation will need to be done.
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 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. Chemotherapy is not very good treatment for adrenal cancer. It is only used to treat adrenal cancer that has become widespread.
Chemotherapy 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.
- 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 chemotherapy 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.
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