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Adrenocortical Carcinoma
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Adrenocortical Carcinoma

(Cancer of the Adrenal Cortex; Adrenal Cortical Carcinoma; Adrenal Cancer)

En Español (Spanish Version)

Definition

The adrenal cortex is the outside layer of the two adrenal glands, which are two hormone-producing glands located just above each kidney. They produce important hormones such as adrenaline, cortisol, and sex hormones (testosterone and estrogen). These hormones regulate heart rate, blood pressure, and many other vital functions.

Cancers of the adrenal cortex are very rare—they make up 0.5-2 cases per million. The majority of these malignant tumors produce excess hormones that can alter regular hormonal balance.

Anatomy of the Adrenal Glands

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© 2008 Nucleus Medical Art, Inc.

Causes

The cause of adrenocortical carcinoma is unknown.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. There are few known risk factors for cancers of the adrenal cortex; however, the following factors may contribute:

  • Being female
  • Children under 5 years old
  • Adults between 40 to 50 years old
  • A genetic defect may cause adrenocortical cancer in some children, but the majority are non-hereditary

Symptoms

Approximately 40% of adrenocortical carcinoma do not secrete any hormone and therefore do not have any specific symptoms. These are discovered either incidentally or as part of an evaluation of abdominal pain.

With tumors that are hormonally active (ie, “functional”), excess hormones may produce symptoms such as high blood pressure , weakening of the bones, or diabetes . Other conditions that may result from functional tumors of the adrenal cortex include:

  • Cushing’s syndrome (Hypercortisolism)—30% of cases—excess cortisol, the hormone which helps the body respond to stressful situations and infections
  • Conn’s syndrome (Hyperaldosteronism)—2% of cases—excess aldosterone, the hormone which helps the body maintain normal levels of sodium and potassium
  • Virilization (20% of cases)—women with functional tumors that release males hormones. Women may experience changes such as a deepening voice, hirsutism (growing excess hair on the face) , and swelling of the sex organs or breasts may occur
  • A mixed Cushing’s syndrome and virilization accounts for 35% of all cases
  • In young children with functional tumors that release sex hormones, these tumors may cause early onset of puberty

These symptoms may be caused by other health conditions. Anyone experiencing these symptoms should see a doctor.

Diagnosis

Your doctor will ask about your symptoms, medical history, and family history, and perform a physical exam.

Tests may include the following:

  • Blood tests to look for hormones from the adrenal glands
  • Urine tests (24 hour cortisol)
  • CT scan of the abdomen —a type of x-ray that uses a computer to make pictures of structures inside the abdomen
  • MRI of the abdomen —a test that uses magnetic waves to make pictures of structures inside the abdomen
  • X-rays

Once cancer of the adrenal cortex is confirmed, you will be referred to an oncologist (cancer doctor) who will do further testing to determine what stage (1-4) the cancer has reached. The stage of a tumor is determined by its size and how far it has spread from its point of origin. At the time of diagnosis, 30%-85% of patients are found to have distant metastasis (when cancer has spread). The higher the stage, the more dangerous and difficult it is to treat a tumor.

Treatment

Treatment of adrenocortical carcinoma depends on the stage of the tumor and your overall health. Talk with your doctor about the best treatment plan for you.

The following methods are used to treat cancer of the adrenal cortex:

Surgery to Remove the Tumor

Surgery is the first treatment approach for most adrenocortical cancers in approximately 75 % of all cases. Your surgeon may remove the adrenal tumor in a surgery known as adrenalectomy . He or she will also need to remove any surrounding tissues or lymph nodes that contains cancer cells.

Radiation Therapy

Radiation therapy uses powerful x-rays to kill cancer. However, radiation therapy is not commonly used to treat adrenal cancer because x-rays are not particularly effective in killing this type of cancer. Its use is limited to treat those with cancer spread to the bones.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer. These drugs may be taken in pill form or be injected directly into a vein or muscle. Chemotherapy is normally only used to treat adrenal cancer that has become widespread or in those with a high risk of recurrence. Overall, it is not particularly effective in treating adrenal cancer.

Other Therapies

A drug called mitotane is the most frequently prescribed drug for people with adrenal cortical cancer. Mitotane blocks hormone production by the adrenal gland and also destroys adrenal cancer cells, but it does have serious side effects. Mitotane is especially helpful in treating functional (hormonally active) tumors of the adrenal cortex. Other hormone production-blocking drugs may also be prescribed if mitotane does not work.

Clinical trials (studies) of new drug and radiation therapies, including gene and immunotherapy, designed to better treat cancer are constantly under way, so your doctor may advise you to participate in one of these trials.

If treatment is successful and all cancerous cells are removed or destroyed, you will still need to be screened for reoccurrence of the cancer on a periodic basis.

Prevention

Since the cause of adrenal cortical cancers is unknown, there are no known preventive measures. Healthy lifestyle choices, however, may reduce your risk for cancers of all types.

RESOURCES:

American Cancer Society
http://www.cancer.org/docroot/home/index.asp

National Cancer Institute
http://www.cancer.gov

CANADIAN RESOURCES:

BC Cancer Agency
http://www.bccancer.bc.ca

Canadian Cancer Society
http://www.cancer.ca

References:

Abiven G, Coste J, Groussin L, et al. Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endo & Metab . 2006;91:2650-55.

Adrenal cortical carcinoma. DynaMed website. Available at: http://dynamed102.epnet.com/Detail.aspx?id=116754. Accessed November 30, 2006.

Adrenocortical carcinoma. National Cancer Institute website. Available at: http://www.cancer.gov/cancertopics/types/adrenocortical . Accessed November 30, 2006.

Adrenocortical carcinoma: treatment statement for health professionals. National Cancer Institute website. Available at: http://www.meb.uni-bonn.de/cancer.gov/CDR0000062907.html . Accessed Nov. 30, 2006.

Allolio B, Fassnacht M. Adrencortical carcinoma: clinical update. J Clin Endocrinol Metab .2006;91:2027-37.

Detailed guide: adrenal cortical cancer. American Cancer Society website. Available at: http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=71 . Accessed November 29, 2006.

Kirschner LS. Review: emerging treatment strategies for adrnocortical carcinoma: a new hope. J Clin Endocinol Metab .2006;91:14-21.

Van Ditzhuijsen cI, van de Weijer R, Haak HR: Adrenocortical carcinoma. Neth J Med. 2007;65:55-60.



Last reviewed February 2008 by David Juan, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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