Treatments for adrenocortical carcinoma (ACC)

The following are treatment options for adrenocortical carcinoma (ACC). The treatments used depend mainly on whether the cancer can be completely removed with surgery (is resectable) or can’t be completely removed with surgery (is unresectable). Stage 1, stage 2 and stage 3 ACCs are often resectable. Unresectable adrenal gland cancers include stage 4 ACC that has spread to distant organs (metastasized).

Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.

Surgery is the main treatment for resectable ACC. You may also be offered surgery for unresectable or recurrent ACC.

Adrenalectomy is a surgery to remove the adrenal gland.It is 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 (surgery for metastasis) may be required. For example, if the cancer has spread to the capsule of the kidney, the kidney is removed. Lymph nodes are also removed if they are enlarged.

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.

You may be offered supportive drugs for ACC. Supportive drugs may be used to control symptoms from high levels of different adrenal gland hormones. These symptoms can severely affect your quality of life.

Cushing syndrome, which is caused by high levels of cortisol, is one of the more common group of symptoms in ACC tumours. Less often, symptoms are caused by high levels of the aldosterone hormone (Conn’s syndrome) or high levels of sex hormones (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 – decreases production of the steroid hormone cortisol
• spironolactone (Aldactone) – blocks the effects of aldosterone

Supportive drugs may also be given to replace adrenal gland hormones that are at very low levels after surgery. This is called hormone replacement therapy. The drugs are given until hormone levels return to normal, which is usually within 2 weeks after surgery. Sometimes hormone replacement therapy is needed permanently.

Chemotherapy is the main treatment for unresectable ACC. It may also be offered after surgery for resectable ACC if the tumour is large or was not completely removed with 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 too much production of certain hormones.

The most common chemotherapy drug used to treat ACC is mitotane (Lysodren). 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 be combined with other chemotherapy drugs such as:

• cisplatin (Platinol AQ)
• doxorubicin (Adriamycin)
• etoposide (Vepesid, VP-16)
• paclitaxel (Taxol)
• 5-fluorouracil (Adrucil, 5-FU)
• vincristine (Oncovin)

Radiation therapy is not used very often for ACC. External beam 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 beam radiation therapy is sometimes used to treat it.

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.

A few clinical trials in Canada are open to people with ACC. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.