What is adrenal cortical cancer?

Adrenal cortical cancer is also known as adrenocortical carcinoma. It is a type of adrenal gland tumour.

There are 3 types of adrenal gland tumours:

  • adrenal cortical cancer (ACC)
  • phaeochromocytoma (pronounced fey-oh-cromo-sy-toma)
  • adenomas. These are not cancer, they are benign tumours. 

This information is about ACC. We also have information about phaeochromocytoma.

Secondary cancer in the adrenal glands

Secondary cancer in the adrenal glands is more common than a cancer that starts in them. A secondary cancer is one that starts in one part of the body and spreads to another. So a secondary adrenal cancer is one that has started elsewhere and spread to the adrenal glands.

For example, kidney cancer can spread to the adrenal glands. Doctors call this secondary kidney cancer. If this happens, you have the same treatment as kidney cancer, not adrenal gland cancer. This means the treatment you need will depend on where the cancer started.

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 2 layers:

  • the outer layer or cortex
  • the inner layer or medulla
Diagram showing the position of the adrenal cortex and adrenal medulla

Tumours can start in either of these areas. Adrenal cortical tumours start in the cortex.

What hormones do adrenal glands make?

The cortex layer of the adrenal gland makes important hormones. The hormones include:

  • cortisol which helps control the blood pressure, blood sugar levels, immune system Open a glossary item and stress response
  • aldosterone which helps to balance the levels of salt and water in the body
  • sex hormones such as oestrogen and testosterone

The medulla layer of the adrenal gland makes hormones that are important for the flight and fight reaction. These hormones help us deal with pressure or ‘threats’. This includes the hormones adrenaline and noradrenaline.

Risks and causes of ACC

It is not known what causes most ACC.

A small number of people are born with gene Open a glossary item changes that increase their risk of developing ACC. These inherited gene changes are rare. They include:

  • Li Fraumeni syndrome
  • Lynch syndrome
  • Beckwith-Wiedemann syndrome
  • familial adenomatous polyposis
  • multiple endocrine neoplasia type 1 (MEN1)
  • carney complex

Symptoms of ACC

Some ACCs make hormones. Doctors call these functioning tumours. Some ACC tumours don’t make hormones. They are non functioning tumours.

You are more likely to have symptoms if your cancer makes hormones. The symptoms you have depends on the type of hormone the tumour produces.

Possible symptoms if your cancer makes hormones

Having too much cortisol in your body can cause a group of symptoms known as Cushing’s syndrome. You might have the following symptoms if you have too much cortisol or aldosterone in your body:

  • swelling of the face giving a rounded appearance
  • a build up of fat on the base of the neck and shoulders
  • muscle wasting and weakness in the legs and arms, making them look thinner
  • weight gain that is more noticeable in the trunk of the body
  • high blood pressure
  • thinning of the skin
  • bruising easily
  • stretch marks which might have a purple colour
  • flushing of the face

You might have the following symptoms if your cancer makes too many sex hormones:

  • women might have a deepening of the voice, changes in periods, loss of hair or growth of facial hair
  • men may have loss of sex drive (libido), impotence and sometimes swelling of the breasts

Possible symptoms if your cancer doesn’t make hormones

Cancers that don't make hormones might not cause symptoms until they are advanced. Possible symptoms include:

  • pain in your tummy (abdomen)
  • tiredness
  • weight loss
  • feeling or being sick

How common is ACC?

ACC is rare. In the UK, around 220 people are diagnosed with adrenal gland cancer every year.

Adrenal gland cancer includes ACC and phaeochromocytoma.

Adenoma of the adrenal glands

An adenoma is a benign tumour. This means that adenomas are not cancer. They are a type of tumour that can also start in the outer layer of the adrenal glands (the cortex).

Adenomas are often found by chance when people are having scans for other reasons. Doctors sometimes call them adrenal incidentaloma.

Most adenomas do not make hormones. They are non functioning tumours. But some do make adrenal hormones such as cortisol and aldosterone. These are functioning tumours or hormone secreting tumours. When the tumour produces lots of these hormones it can cause symptoms.

You might not need treatment if you have an adenoma. You usually only need it if the tumour is growing or making large amounts of hormones.

Diagnosing ACC

You usually need several tests if your doctor thinks you have an ACC. You will have blood and urine tests and scans such as CT and MRI scans.

  • Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN clinical practice guidelines for diagnosis, treatment and follow up
    M Fassnacht and others
    Annals of Oncology, 2020. Vol 31, Issue 11. Pages 1476-1490

  • Cancer: Principles and Practice of Oncology (10th edition)
    VT DeVita, TS Lawrence, SA Rosenberg
    Lippincott, Williams and Wilkins, 2015

  • The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors
    CA Koch and others
    The Journal of Clinical Endocrinology & Metabolism, 2002. Vol 87, Issue 12. Pages 5367-5384

  • Adrenocortical carcinoma: clinical update
    B Allolio and M Fassnacht
    The Journal of Clinical Endocrinology & Metabolism, 2006. Vol 91, Issue 6. Pages 2027-2037

  • Cancer incidence from the National Cancer Registration and Analysis Service (NCRAS) (2017-2019 England average)
    Last accessed Dec 2021

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact patientinformation@cancer.org.uk with details of the particular issue you are interested in if you need additional references for this information.

Last reviewed: 
25 Oct 2022
Next review due: 
25 Oct 2025

Related links