Understanding adrenocortical carcinoma (ACC)
Adrenocortical carcinoma (ACC) is a rare aggressive form of cancer that grows in the outer part of the adrenal glands.2
What is Adrenocortical Carcinoma (ACC)?

The adrenal glands sit just above the kidneys and produce hormones, including cortisol, aldosterone and male sex hormones.1 Tumours in these glands are common, but ACC is a rare aggressive form of cancer that grows in the outer part of the glands. The tumours in the adrenal glands cause too much of multiple types of hormone to be produced.2,3

How common is ACC?
ACC is rare – there are only 0.7–2.0 new cases per million people each year.4

ACC can occur at any age, but it is more common in children younger than 10 years of age and in adults between 40 and 50 years. Women are more frequently affected than men.3 Family history of certain inherited diseases and having certain genes can make it more likely you will develop ACC.2

What are the symptoms of ACC?
Symptoms include excess hair growth on the face, chest and back.
As well as nausea/vomiting, muscle weakness, back and abdominal pain, passing water more often, weight gain/weight loss, high blood pressure, and high sugar levels.2,3
How is ACC diagnosed?
ACC can be difficult to diagnose, because the symptoms are also seen in more common diseases.
The tests used to diagnose ACC depend on the patient’s symptoms, but usually begin with a simple check of the outward signs of the disease. These can include excess hair growth and high blood pressure. More extensive tests, such as measuring the hormone levels in the blood, imaging (including CT and MRI) and a microscopic examination of gland tissue are also carried out. These tests allow the doctor to see how far the tumour has developed and how best to treat the disease.1,2
What treatment options are there for ACC?
Treatment aims to reduce cortisol levels back to normal.
Surgery to remove the affected adrenal gland is usually the first choice of treatment. Medicines, or radiotherapy, aim to reduce the risk of recurrence. 2,3,5
If you are a patient, please contact your physician for more information.
If you are a healthcare professional from the EU, you can find further information on the treatment guidelines for adrenocortical carcinoma here.
Want more information about ACC?
You can visit the following website: www.adrenalcorticalcarcinoma.com
If you are looking for additional information or support the following patient associations will be able to help you.
HRA Pharma Rare Diseases is not responsible for the nature, content and availability of external websites
References
1. Słapa RZ et al. J Ultrason. 2015;15:377–387
2. Else T et al. Endocr Rev. 2014;35:282–326
3. Libe R et al. Front Cell Div Biol. 2015;3:45
4. Fassnacht M et al. Eur J Endocrinol. 2018;179:G1–G46
5. Waszut U et al. J Physiol Pharmacol. 2017;68:13–26