Adrenocortical Carcinoma

Tumor - adrenal

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. In women the tumor often releases hormones. Adrenocortical carcinoma can produce the hormones cortisol or aldosterone.

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

Symptoms that suggest increased cortisol production:

• Moon face: Flushed rounded face with pudgy cheeks
• Obesity
• Buffalo hump: rounded fatty hump high on the back just below the neck
• Short stature: stunted growth in height, may be less than 5th percentile
• Virilization: increased body hair (hypertrichosis) especially on face, pubic hair, acne, deepening of voice, enlarged clitoris (girls)

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

• High blood pressure
• High serum cortisol levels
• Low serum ACTH
• Low serum potassium
• High serum aldosterone
• CT scan of the abdomen
• Abdominal x-ray
• Abdominal MRI

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). Metastatic tumors are often fatal within a few years.

A complication is metastasis (often to the liver, bone, and lung).

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

Abeloff M, Armitage J, Niederhuber J, Kastan M, McKenna WG. Clinical Oncology. 3rd ed. Philadelphia, Pa: Churchill Livingstone, 2004.

Assié G, Antoni G, Tissier F, et al. Prognostic parameters of metastatic adrenocortical carcinoma. J Clin Endocrinol Metab. January 2007;92:148-154.

Allolio B, Fassnacht M. Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab. June 1, 2006;91:2027-2037.