Adrenocortical carcinoma

Endocrine glands

Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

Also known as: Tumor - adrenal

Definition

Adrenocortical carcinoma is a cancer of the adrenal glands.

Causes, incidence, and risk factors

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, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

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

Symptoms

Symptoms that suggest increased cortisol or other adrenal gland hormone 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 the 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:

  • Increased thirst
  • Muscle cramps
  • Urination
  • Weakness

Signs and tests

A physical exam may reveal high blood pressure and changes in body shape, such as breast enlargement in men (gynecomastia) or male characteristics in women (virilization). Blood tests will be done to check hormone levels:

Imaging tests may include:

Treatment

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.

Expectations (prognosis)

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.

Complications

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

Calling your health care provider

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

References

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

National Cancer Institute. Adrenocortical carcinoma treatment PDQ. Updated May 16, 2008.

See also:

  • Special Topic

    Adrenal glands

    Endocrine glands

    Adrenal glands are triangle-shaped glands located on top of the kidneys. The outer part of the adrenal gland is called the cortex and produces steroid hormones such as , , and . The inner part of the adrenal gland is called the medulla and produces and , which are commonly called adrenaline and ...

  • Disease

    Tumor

    A tumor is an abnormal growth of body tissue. Tumors can be cancerous (malignant) or noncancerous (benign).

  • Special Topic

    Metastasis

    Lymph node metastases, CT scan

    Metastasis is the movement or spreading of cancer cells from one organ or tissue to another. Cancer cells usually spread through the bloodstream or the .

Review date:
August 14, 2010
Reviewed by:
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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