Adrenocortical carcinoma

Adrenal metastases, CT scan

Adrenal Tumor - CT

Adrenal tumor, intravenous pyelogram (IVP)

Adrenocortical carcinoma

Definition:

Adrenocortical carcinoma is a malignant tumor of the adrenal glands.

Alternative Names:

Tumor - adrenal

Causes, incidence, and risk factors:

The cause is unknown. Adrenal cortical carcinoma may be associated with an inherited cancer syndrome. Both men and women develop this tumor, but in women it is more often functional (secreting hormones).

Adrenal cortical carcinomas can produce the hormones cortisol or aldosterone. The disease is most common in two age groups: young children less than 5 years old, and adults in their 30s and 40s. The incidence is about 2 per million.

Symptoms:

Findings suggestive of increased cortisol production:

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

Findings suggestive of increased aldosterone production are symptoms of low potassium (weakness, muscle cramps, increased thirst, and urination).

Signs and tests:

High blood pressure
High serum cortisol levels
Low serum ACTH
Low serum potassium
High serum aldosterone
CT scan of the abdomen (may demonstrate a mass in the adrenal gland)
Abdominal X-ray
Abdominal MRI (may demonstrate a mass in the adrenal gland)

Treatment:

Primary treatment consists of surgical removal of the tumor. Adrenocortical carcinoma may not respond well to chemotherapy. Medications may be given to reduce production of cortisol which is responsible for many of the symptoms.

Expectations (prognosis):

The outcome is dependent on how early the diagnosis is made and whether the tumor has spread (metastasized). These tumors are often fatal within a few years.

Complications:

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

Calling your health care provider:

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

Review Date: 10/13/2001
Reviewed By: Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2003 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.