• Adrenocortical Carcinoma

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

    Definition

    Adrenocortical carcinoma is cancer of the adrenal cortex. The adrenal glands are two glands located just above each kidney. The glands produce important hormones. These hormones regulate heart rate, blood pressure, and many other vital functions. The adrenal cortex is the outside layer of the two adrenal glands.
    Cancers of the adrenal cortex are very rare. It occurs in 0.5-2 cases per million. The majority of these tumors produce excess hormones. The excess hormones can change hormonal balance.
    Anatomy of the Adrenal Glands
    nucleus factsheet image
    Copyright © Nucleus Medical Media, Inc.

    Causes

    The cause is unknown.

    Risk Factors

    There are few known risk factors for cancers of the adrenal cortex. But, the following factors may contribute:
    • Sex: 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 of cases are non-hereditary

    Symptoms

    Approximately 40% of adrenocortical carcinoma do not secrete any hormone. These people do not have any specific symptoms. Adrenocortical carcinoma is discovered either by accident or as part of a physical examination for abdominal pain.
    Other tumors are hormonally active (functional). Excess hormones may produce symptoms such as:
    Other conditions that may result from functional tumors of the adrenal cortex include:
    • Cushing’s syndrome (Hypercortisolism)—30% of cases—excess cortisol which helps the body respond to stressful situations and infections
    • Conn’s syndrome (Hyperaldosteronism)—2% of cases—excess aldosterone, which helps the body maintain normal levels of sodium and potassium
    • Virilization (20% of cases)—women with functional tumors that release males hormones; may deepen voice, cause 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, and medical and family history. A physical exam will be done.
    Tests may include the following:
    After cancer of the adrenal cortex is confirmed, you will be referred to an oncologist. This type of doctor focuses on cancer.
    Additional testing will be done 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 cancer that has spread. The higher the stage, the more dangerous and difficult it is to treat.

    Treatment

    Treatment depends on the stage of the tumor and your overall health. Talk with your doctor about the best plan for you.
    The following methods are used to treat cancer of the adrenal cortex:

    Adrenalectomy Surgery to Remove the Tumor

    Surgery to remove the adrenal tumor is the first treatment approach for about 75 % of all cases. The doctor will also need to remove any surrounding tissues or lymph nodes that contain cancer cells.

    Radiation therapy and Chemotherapy

    These treatments tend to be less effective in treating this type of cancer. They are most often used if the cancer has spread. Chemotherapy may also be used if there is a high chance the cancer will come back.

    Other Therapies

      Mitotane is the drug most often used for this type of cancer. It blocks hormone production by the adrenal gland. It also destroys adrenal cancer cells. This drug does have serious side effects.
      • Especially helpful in treating functional tumors of the adrenal cortex
    • Other hormone blocking drugs may be given if mitotane does not work.
    • Research of new drug and radiation therapies are under way. This includes gene and immunotherapy. Your doctor may advise you to participate in one of these trials.
    If treatment is successful, you will still need to be screened for reoccurrence of the cancer on a periodic basis.

    Prevention

    There are no known preventive measures. Healthy lifestyle choices may reduce your risk for cancers of all types.

    RESOURCES

    American Cancer Society http://www.cancer.org/

    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. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated August 8, 2012. Accessed October 31, 2012.

    Adrenocortical carcinoma. National Cancer Institute website. Available at: http://www.cancer.gov/cancertopics/types/adrenocortical . Accessed October 31, 2012.

    Adrenocortical carcinoma: treatment statement for health professionals. National Cancer Institute website. Available at: http://www.meb.uni-bonn.de/cancer.gov/CDR0000062907.html . Updated October 23, 2012. Accessed October 31, 2012.

    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%5F2%5F3x.asp?dt=71 . Accessed October 31, 2012.

    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.

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