Adrenocortical carcinoma (ACC) is a rare aggressive form of cancer that grows in the outer part of the adrenal glands.1
What is Adrenocortical Carcinoma (ACC)?1
The adrenal glands sit just above the kidneys and produce hormones, including cortisol, aldosterone and male sex hormones. Tumors in these glands are common, but ACC is a rare aggressive form of cancer that grows in the outer part of the glands. The tumors in the adrenal glands cause too much of multiple types of hormone to be produced.
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?
Some patients with ACC don’t have symptoms, while others do. Symptoms can mimic other conditions, which makes ACC difficult to diagnose. Up to 20% of patients are diagnosed by accident.3
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.1
How is ACC diagnosed?1
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 tumor has developed and how best to treat the disease.
What treatment options are there for ACC?1
Surgery to remove the affected adrenal gland is usually the first choice of treatment. Medicines, or radiation therapy aim to reduce the risk of recurrence.
If you are a patient, please contact your physician for more information.
- Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoili EM, Jolly S, Miller BS, Giordano TJ, Hammer GD. Adrenocortical carcinoma. Endocr Rev. 2014 Apr;35(2):282-326
- Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment. Front Cell Dev Biol. 2015 Jul 3:45
- Fassnacht M, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018;179(4):G1-G46
Additional Resources for adrenocortical carcinoma:
- Unlocking an ACC Diagnosis (pdf)
- ACC animation + Lysodren
- Navigating an Adrenocortical Carcinoma Diagnosis
- National Cancer Institute (link)
- ACC Infographic (pdf)
- Clinical Management of Adrenocortical Carcinoma (pdf)
- Clinical Management of Adrenocortical Carcinoma (video)
- Disease State Clinical Review on the Evaluation and Management of Adrenocortical Carcinoma in an Adult: A Practical Approach
Prognosis for ACC when treated:
The 5-year survival rate for ACC depends greatly on when the tumor is found.
5-year stage-dependent survival %
- Stage 1
- Stage 2
- Stage 3
- Stage 4
- Stage 1
- Stage 2
- Stage 3
- Stage 4
Fassnacht M, et al. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer. 2009;115(2):243-50.