Adrenocortical carcinoma is a cancer of the adrenal glands.

Adrenocortical carcinoma is most common in children younger than 5 and adults in their 30s and 40s.

Adrenocortical carcinoma 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, estrogen, or aldosterone. In women the tumor often releases the hormones, which can lead to male characteristics.

The cause is unknown. About 2 people per million develop this type of tumor.

Symptoms that suggest increased cortisol production:

• Fatty, rounded hump high on the back just below the neck (buffalo hump)
• Flushed rounded face with pudgy cheeks (moon face )
• Obesity
• Stunted growth in height (short stature)
• Virilization - the appearance of male characteristics, including increased body hair especially on face, pubic hair, acne, deepening of voice, and enlarged clitoris (girls)

Symptoms that suggest increased aldosterone production are the same as symptoms of low potassium and include weakness, muscle cramps, increased thirst, and urination.

A physical exam reveals high blood pressure. Blood work 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.

Imaging tests may include:

• Abdominal MRI
• Abdominal x-ray
• CT scan of the abdomen

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 often lead to death within 1 to 3 years.

The tumor can spread to the liver, bone, lung, or other areas.

Call your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.

Lal G, O’Dorisio T, McDougall R, Weigel RJ. Cancer of the endocrine system. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKena WG, eds. Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 75.