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Adrenocortical carcinoma

Tumor - adrenal; ACC - adrenal

Adrenocortical carcinoma (ACC) is a cancer of the adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.

Causes

ACC is most common in children younger than 5 years old and adults in their 40s and 50s.

The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.

ACC 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.

ACC is very rare. The cause is unknown.

Symptoms

Symptoms of increased cortisol or other adrenal gland hormones may include:

  • Fatty, rounded hump high on the back just below the neck (buffalo hump)
  • Flushed, rounded face with pudgy cheeks (moon face)
  • Obesity
  • 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
  • Weakness
  • Pain in the abdomen 

Exams and Tests

 The health care provider will perform a physical exam and ask about your symptoms.

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 of the abdomen may include:

  • Ultrasound
  • CT scan
  • MRI
  • PET scan

Treatment

Primary treatment is surgery to remove the tumor. ACC may not improve with chemotherapy. Medicines may be given to reduce production of cortisol, which causes many of the symptoms.

Outlook (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.

Possible Complications

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

When to Contact a Medical Professional

Call your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.

References

Allolio B, Fassnacht M. Adrenocortical carcinoma. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 107.

National Cancer Institute. PDQ: adrenocortical carcinoma treatment - health professional version. Updated June 02, 2015. Cancer.gov. www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq#section/all. Accessed October 17, 2016.

    • Endocrine glands

      Endocrine glands - illustration

      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 rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

      Endocrine glands

      illustration

    • Adrenal metastases, CT scan

      Adrenal metastases, CT scan - illustration

      This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.

      Adrenal metastases, CT scan

      illustration

    • Adrenal Tumor - CT

      Adrenal Tumor - CT - illustration

      CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.

      Adrenal Tumor - CT

      illustration

      • Endocrine glands

        Endocrine glands - illustration

        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 rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

        Endocrine glands

        illustration

      • Adrenal metastases, CT scan

        Adrenal metastases, CT scan - illustration

        This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.

        Adrenal metastases, CT scan

        illustration

      • Adrenal Tumor - CT

        Adrenal Tumor - CT - illustration

        CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.

        Adrenal Tumor - CT

        illustration

      Review Date: 8/15/2016

      Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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