Adrenal cortical cancer
There are 2 adrenal glands, one above each kidney. Adrenal means next to the kidney.
The adrenal glands have an inner and outer layer. The outer layer is the cortex and the inner the medulla. Cancer can start in either of these areas of the adrenal gland. This page is about adrenal cortical tumours, including adrenal cortical carcinoma (ACC). Cortical means the cells of the cortex. Carcinoma means cancer.
If you are looking for information about cancer that started in the inner part of the adrenal gland (the medulla), this is not the right page for you. Medullary adrenal cancer is called phaeochromocytoma.
We have a separate page about phaeochromocytoma.
The adrenal glands are small, but very important because they make several hormones that help the body to work properly and are vital to life. The most common type of tumour found in the adrenal cortex is adenoma. Adenomas are non cancerous (benign) tumours. They are often found by chance in patients having scans for other reasons. When benign tumours are found by chance doctors call them incidentalomas. Most adenomas do not make steroid hormones (they are non functioning tumours). But some make too much of these hormones (functioning or hormone secreting tumours). The excess steroid hormones can cause symptoms. Adenomas may not need any treatment unless they are growing or making large amounts of steroid hormones.
This page is about cancers that start in the adrenal gland. They are called adrenal cortical carcinomas (ACC) or primary adrenal gland cancers. They are very rare. More commonly, cancers found in the adrenal glands started somewhere else in the body, such as the kidney, and spread there. This is known as secondary cancer and would be treated as kidney cancer for example, not an adrenal gland cancer. If you have cancer that has spread to the adrenal glands, this is not the right page for you. You need to go to the section about your primary cancer.
Adrenal cortical cancer can happen at any age, but is more likely under the age of 5 and in adults in their 40s and 50s. Up to 10 to 15 out of 100 ACC's (10 to 15%) are linked to rare family cancer syndromes, where an inherited change in a gene (mutation) causes a number of cancers to develop within a family. These syndromes include Li Fraumeni syndrome, Lynch syndrome and multiple endocrine neoplasia type 1 (MEN1).
The adrenal cortex makes steroid hormones. Benign or cancerous (malignant) adrenal cortical tumours may make too much of any of these hormones. The hormones cause symptoms that affect your whole body. The hormones include
- Cortisol – a natural steroid hormone that affects the level of sugar in the blood
- Aldosterone - helps to regulate the body’s water balance, salt balance and blood pressure
- Androgen hormones - these hormones are changed elsewhere in the body to female hormones (oestrogens) and male hormones (androgens). These hormones are made in much larger amounts by the ovaries in women and the testes in men.
About 2 out of 3 adrenal cortical cancers make excessive amounts of hormones. If you have one that doesn't, you may not have any symptoms until the cancer is quite far advanced. Then you may start to have pain, tiredness and weight loss.
If your tumour makes hormones, these will cause your symptoms. If your tumour makes too much cortisol or aldosterone, you may have
- Raised blood pressure
- Passing urine frequently
- Muscle cramps
- Weight gain that is more noticeable in the trunk of the body
- Muscle wasting in the legs or arms, making them look thinner
- Fat accumulation in the base of the neck (known as a buffalo hump)
- Swelling of the face giving a rounded appearance
- Mild diabetes (less common)
- Abnormal hair growth on the face, arms and upper back
Do bear in mind there are many other causes for raised blood pressure which are far more common than adrenal gland cancer.
If your tumour makes too much sex hormones
- Women may have deepening of the voice, changes in periods, baldness or growth of facial hair
- Men may have loss of sex drive (libido), impotence and sometimes swelling of the breasts
Unfortunately, adrenal cortical carcinoma can be quite a fast growing type of cancer. They are often diagnosed in the later stages (when they are quite advanced) and this means it can be difficult to cure them. Treatment depends on the size of the tumour and whether it has spread, as well as your general health and fitness. Treatment includes
If your cancer is diagnosed early enough, surgery to remove the affected adrenal gland is usually the first choice of treatment and can cure the cancer. This operation is called an adrenalectomy. If your surgeon thinks the cancer may have spread locally, they will remove the tissues immediately surrounding the adrenal gland and nearby lymph nodes.
Hormones produced by the cancer can make your blood pressure unstable so it can change suddenly during and immediately after the operation. To make sure it is as stable as possible, your doctor may give you blood pressure medicines from around 10 days before the operation. After surgery, your blood pressure should go back to normal.
If you need to have one of your adrenal glands removed, your other one will carry on making all the hormones you need. If you have both adrenal glands removed, you will have to take hormone replacement tablets every day for the rest of your life.
In some cases, your doctor may suggest surgery for ACC that has spread. This is usually if there are only 1 or 2 small well contained areas.
Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. You may have just one drug or a combination of more than one drug. Doctors usually use chemotherapy to treat ACC that has come back or spread to other parts of the body.
The most common drug doctors use is mitotane (Lysodren). As well as destroying the cancerous adrenal cells, it also blocks the production of hormones by the tumour. This means doctors may use mitotane to help control troublesome symptoms caused by excess hormones.
In some cases, you may have mitotane after surgery to reduce the chances of the cancer coming back.
Some of the other chemotherapy drugs used to treat adrenal cortical cancer are
- Doxorubicin (Adriamycin)
- Paclitaxel (Taxol)
- Fluorouracil (5FU)
- Etoposide (VP16
You usually have 2 or more of these drugs together. For example etoposide, doxorubicin and cisplatin. You may have this combination of drugs with mitotane. Click on the links above to find out about the specific side effects of these drugs.
We have more information about having chemotherapy.
Doctors most often use external radiotherapy for ACC that has spread. It can be very useful for shrinking cancer that has spread to the bones.
You may have radiotherapy for earlier stage adrenal cortical cancer after surgery. But this is still an experimental treatment, so you are most likely to have it as part of a clinical trial.
We have more information about radiotherapy.
Researchers and doctors around the world are continually trying to improve treatments for adrenal cortical cancer. But because this type of cancer is so rare, it makes it difficult to carry out trials.
An international trial called ADIUVO is looking at how well mitotane works after surgery for people with ACC. The people taking part have a low risk of the cancer coming back. Doctors do not know if it's better to give this group of patients mitotane or closely monitor them.
Researchers are also looking to see if different types of biological therapies are helpful for ACC. There have been mixed results so far.
You can find information about UK clinical trials on our clinical trials database.
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