Adrenocortical carcinoma is a cancer of the adrenal glands.
Causes of Adrenocortical carcinoma
Adrenocortical carcinoma is most common in children younger than 5 years old and adults in their 30s and 40s.
The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.
Adrenocortical carcinoma can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.
Adrenocortical carcinoma is very rare. The cause is unknown. About 2 people per million develop this type of tumor.
Adrenocortical carcinoma Symptoms
Symptoms of increased cortisol or other adrenal gland hormones:
- Fatty, rounded hump high on the back just below the neck (buffalo hump)
- Flushed, rounded face with pudgy cheeks (moon face)
- Stunted growth (short stature)
- Virilization - the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris (girls)
Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:
- Muscle cramps
Tests and Exams
The doctor or nurse will examine you. You may have high blood pressure and changes in body shape, such as breast growth in men (gynecomastia) or male signs in women (virilization). Blood tests will be done to check hormone levels:
- ACTH level will be low.
- Aldosterone level will be high.
- Cortisol level will be high.
- Potassium level will be low.
- Male or female hormones may be abnormally high.
Imaging tests may include:
Treatment of Adrenocortical carcinoma
Primary treatment is surgery to remove the tumor. Adrenocortical carcinoma may not improve with chemotherapy. Medications may be given to reduce production of cortisol, which causes many of the symptoms.
The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.
The tumor can spread to the liver, bone, lung, or other areas.
When to Contact a Health Professional
Call your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.
National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine tumors. 2012. Version 1.2012.
Nieman LK. Adrenal cortex. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 234.
|Review Date: 8/31/2014
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.