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About adrenal cortical cancer

Adrenal cortical cancer (ACC) is very rare. It starts in the outer layer of the adrenal glands, which are next to the kidneys. The adrenal glands make hormones. ACC can make too much of these hormones which may cause symptoms that affect your whole body.

The adrenal glands

There are 2 adrenal glands, one above each kidney. Adrenal means next to the kidney.

Diagram showing the position of the adrenal glands

The adrenal glands are small, but very important. They make hormones that help the body work properly and are vital to life.

The adrenal glands have an inner and outer layer: 

  • cortex - outer layer
  • medulla - inner layer

Tumours of the adrenal glands can start in either of these areas.

Tumours that start in the cortex are called adrenal cortical tumours. This page is about adrenal cortical tumours, including adrenal cortical carcinoma (ACC). 

Adrenal tumours that start in the medulla are called phaeochromocytoma (fey-oh-cromo-sy-toma).

Steroid hormones

The adrenal cortex makes steroid hormones. Non cancerous (benign) or cancerous (malignant) adrenal cortical tumours can make too much of any of these hormones. The hormones may cause symptoms that affect your whole body. The hormones include:

  • cortisol – a hormone that can upset the level of sugar in the blood
  • aldosterone - a hormone that 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)

Androgen hormones are made in much larger amounts by the ovaries in women and the testes in men.

Cancers that spread to the adrenal glands

Cancer can start to grow elsewhere in the body and spread to the adrenal glands. This is secondary cancer. Secondary cancer in the adrenal glands is more common than cancer that starts in the adrenal glands.

For example, kidney cancer can spread to the adrenal glands. This is known as secondary cancer and would be treated as kidney cancer, not an adrenal gland cancer.

You should look for information about your primary cancer type if you cancer has spread to the adrenal glands.  

Cancers that start in the adrenal cortex

There are 2 main types of tumour that start in the adrenal cortex.

Adenoma

The most common type of tumour starting in the adrenal cortex is an 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 sometimes call them incidentalomas.

Most adenomas do not make hormones. They are called non functioning tumours. But some make too much of these adrenal hormones or steroid hormones. These are called functioning tumours or hormone secreting tumours. The excess  hormones can cause symptoms.

Adenomas often don't need any treatment unless they are growing or making large amounts of steroid hormones.

Adrenal cortical carcinomas (ACC)

Cancers that start in the adrenal cortex are very rare. They are called adrenal cortical carcinomas (ACC) or primary adrenal gland cancers. Cortical means the cells of the cortex. Carcinoma means cancer. 

Causes

It is not known what causes most ACCs. 

A small number of people are born with an inherited gene change that increases their risk of developing ACC. These inherited syndromes are rare. They include:

  • Li Fraumeni syndrome
  • MEN1 syndrome
  • familial adenomatous polyposis coli
  • congenital adrenal hyperplasia

Adrenal cortical cancers diagnosed in children and young adults are more likely to be lnked to an inherited gene change.

Symptoms

You are more likely to have symptoms if your tumour makes hormones. About 2 out of 3 adrenal cortical cancers make too much of the adrenal or steroid hormones.

If your tumour makes too much cortisol or aldosterone, you might have:

  • raised blood pressure
  • thirst
  • 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
  • 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 hormone:

  • women might 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

Those cancers that don't make any hormones might not cause symptoms until they are quite advanced. In this situation, possible symptoms include:

  • pain
  • tiredness
  • weight loss
Last reviewed: 
20 Jun 2018
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