Treatments for adrenocortical carcinoma (ACC)
The following are treatment options for adrenocortical carcinoma (ACC). Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.
Treatment decisions depend mainly on whether the cancer can be completely removed with surgery (is resectable) or can’t be completely removed with surgery (is unresectable).
Resectable ACC is usually stage 1, stage 2 or stage 3.
Unresectable ACC includes stage 4 ACC that has spread to distant organs (called metastatic ACC).
Surgery
Surgery is a medical procedure to examine, remove or repair tissue. Surgery, as a treatment for cancer, means removing the tumour or cancerous tissue from your body.
Surgery is the main treatment for resectable ACC. You may also be offered surgery for unresectable or recurrent ACC.
An adrenalectomy is a surgery to remove the adrenal gland. It's done if the cancer is only in the adrenal gland. If the cancer has spread to other organs and it can still be fully removed, more surgery to remove cancerous tissue in the organs (called surgery for metastasis) may be required. For example, if the cancer has spread to a kidney, the kidney is removed. Lymph nodes are also removed if they are enlarged.
An en bloc resection removes the tumour and surrounding tissues as a single piece. The location of the tumour and how far it has grown into surrounding tissues determine the amount of tissue that is removed. It may be done for ACC that has spread to nearby tissues. When ACC has spread to other organs, an adrenalectomy is also done as part of the en bloc resection.
Debulking means removing as much of the cancer as possible. This surgery may be used for unresectable or recurrent ACC. Debulking helps to reduce the symptoms of high hormone levels and symptoms caused by growth of the tumour.
Side effects of surgery will depend mainly on the type and site of surgery and your overall health. Tell your healthcare team if you have side effects that you think are from surgery. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.
Surgery for adrenal gland cancer may cause:
- pain
- less urine than normal
- changes in blood pressure
- a low blood sugar level
Find out more about surgery.
Supportive therapy drugs
Different types of supportive therapy drugs may be offered as treatment for ACC.
Drugs to control symptoms of high levels of adrenal gland hormones may be used. These symptoms can severely affect your quality of life.
Cushing syndrome, which is caused by a high level of cortisol, is one of the more common group of symptoms in ACC tumours. Less often, symptoms are caused by a high level of the aldosterone hormone (called Conn’s syndrome) or high levels of sex hormones (called adrenogenital syndrome).
Supportive drugs are given to lower the amount of hormones produced or to block the actions of these hormones. These drugs include:
- mitotane (Lysodren) – a chemotherapy drug taken as a pill
- ketoconazole – a drug that decreases production of cortisol
- spironolactone (Aldactone) – a drug that blocks the effects of aldosterone
Drugs to replace very low levels of adrenal gland hormones after surgery are also called hormone replacement therapy (HRT) drugs. They are given until hormone levels return to normal. Hormone levels usually return to normal within 2 weeks after surgery if the cancer was completely removed. If both of the adrenal glands are removed, you will need to take HRT for the rest of your life.
Drugs to help prevent bone loss such as bisphosphonates may be given if cancer has spread to the bones. These drugs may also be needed if you have a high level of cortisol in your blood.
Side effects of supportive drugs will depend mainly on the type and dose of the drug. Tell your healthcare team if you have side effects that you think are from supportive therapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.
Chemotherapy
Chemotherapy uses drugs to destroy cancer cells. These drugs target rapidly dividing cells throughout the whole body. This means that chemotherapy kills cancer cells but it can also damage healthy cells.
With most types of chemotherapy, the drugs travel through the blood to reach and destroy cancer cells all over the body, including cells that may have broken away from the primary tumour. This is described as systemic therapy.
Chemotherapy is the main treatment for unresectable ACC. It may also be offered after surgery for resectable ACC if the tumour is large, was not completely removed with surgery or has a high risk of returning after surgery. You may also be offered chemotherapy for recurrent ACC.
Chemotherapy is used to kill cancer cells and sometimes shrink the tumour. It also blocks the tumour from making high levels of hormones and helps reduce symptoms caused by the overproduction of certain hormones.
Mitotane is the most common chemotherapy drug used to treat ACC. It is absorbed by the adrenal cortex and tumours that start there. It is taken by mouth (orally) daily and the dose may be increased based on how well it works and how it affects you.
Mitotane may also be added to the combination of cisplatin, doxorubicin and etoposide.
Side effects of chemotherapy will depend mainly on the type of drug, the dose, how it's given and your overall health. Tell your healthcare team if you have side effects that you think are from chemotherapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.
Some common side effects of chemotherapy drugs used for adrenal gland cancer are:
- low levels of adrenal gland hormones
- low blood cell counts
- nausea and vomiting
- diarrhea
- loss of appetite
- skin problems
- changes in blood pressure
Find out more about chemotherapy.
Radiation therapy
Radiation therapy uses high-energy rays or particles to destroy cancer cells.
Radiation therapy is not used very often for ACC. External radiation therapy may be offered after surgery if the tumour is large or was not completely removed with surgery to help reduce the risk of the cancer coming back.
If ACC spreads to other organs such as the bone or brain (called metastatic cancer), external radiation therapy is sometimes used to treat it.
During radiation therapy, your healthcare team protects healthy cells in the treatment area as much as possible. Side effects of radiation therapy will depend mainly on the size of the area being treated, the specific area or organs being treated, the total dose of radiation and the treatment schedule. Tell your healthcare team if you have side effects that you think are from radiation therapy. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.
Some common side effects of radiation therapy used for adrenal gland cancer are:
Find out more about radiation therapy.
Other treatments
Other treatments may be offered for metastatic and recurrent ACC, including:
- radiofrequency ablation (RFA)
- transarterial chemoembolization (TACE)
- cryosurgery
Side effects will depend mainly on the type of treatment given. Tell your healthcare team if you have side effects that you think are from these treatments. The sooner you tell them of any problems, the sooner they can suggest ways to help you deal with them.
Clinical trials
Talk to your doctor about clinical trials open to people with ACC in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.
If you can’t have or don’t want cancer treatment
You may want to consider a type of care to make you feel better without treating the cancer itself. This may be because the cancer treatments don't work anymore, they're not likely to improve your condition or they may cause side effects that are hard to cope with. There may also be other reasons why you can't have or don't want cancer treatment.
Talk to your healthcare team. They can help you choose care and treatment for advanced cancer.