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Adrenocortical Carcinoma

Last updated March 23, 2018

Approved by: Krish Tangella MD, MBA, FCAP


Low magnification microscopic pathology image showing Adrenocortical Carcinoma. H&E stain.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Adrenal Cortex Cancer
  • Adrenal Cortex Carcinoma
  • Carcinoma of Adrenal Cortex

What is Adrenocortical Carcinoma? (Definition/Background Information)

  • Adrenocortical Carcinoma is a malignant tumor that develops within the cortex (outer portion) of the adrenal glands
  • There are two adrenal glands that are located above the kidneys on both the right and left side of the body. The adrenal gland is divided into two compartments - the outermost layer is called the cortex, and the innermost layer is called the medulla
  • The cortex is the area that is involved in Adrenocortical Carcinoma. The exact cause of this condition is not known
  • The primary function of the adrenal cortex is to produce certain hormones in the body such as aldosterone, cortisol, and androgens. These hormones regulate many physiologic functions of the body
  • Typically, Adrenocortical Carcinoma can affect any individual; there is no preference for any particular age group or gender
  • Common signs and symptoms associated with Adrenocortical Carcinoma include the presence of a mass in the abdomen, pain, and symptoms such as increased facial and pubic hair growth, acne, and changes in one’s voice
  • The primary treatment of Adrenocortical Carcinoma is a surgical removal of the tumor. If the cancer is diagnosed early, before it has spread to other parts of the body, then it has a slightly better prognosis

Who gets Adrenocortical Carcinoma? (Age and Sex Distribution)

  • Adrenocortical Carcinoma can occur in any age group with no gender predilection
  • However, current studies demonstrate a higher occurrence in populations younger than 5 years and adults between the ages of 30 and 40 years
  • Studies have not yet indicated specific evidence indicating that the condition is more predominant in a particular racial or ethnic group

What are the Risk Factors for Adrenocortical Carcinoma? (Predisposing Factors)

Following are the risk factors associated with Adrenocortical Carcinoma:

  • There are certain genetic conditions that increase the risk of developing an Adrenocortical Carcinoma. These conditions are usually present from birth and occur as a syndrome such as Li Fraumeni syndrome and Carney complex
  • Some individuals are at an increased risk due to their positive family history

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Adrenocortical Carcinoma? (Etiology)

The exact cause for Adrenocortical Carcinoma development is still unknown.

  • Studies do not show any correlation of smoking, consumption of alcohol, or environmental factors for the condition to develop
  • A small group of people are, however, affected due to a gene defect. This gene, known as p53, is responsible for many processes within the body. If, for various reasons, the gene becomes abnormal, then it leads to the production of harmful cells that become cancerous to the body

What are the Signs and Symptoms of Adrenocortical Carcinoma?

The common signs and symptoms of Adrenocortical Carcinoma that are observed are:

  • Presence of a mass in the abdomen
  • Abdominal and back pain
  • Virilization: Due to an overproduction of androgens (hormone type), females may present with a deepened voice, acne, excessive hair growth especially on the face, and enlargement of the clitoris. Males may present with breast growth
  • Cushing syndrome: Due to excessive production of the cortisol hormone, individuals may have symptoms of Cushing syndrome that include rounded moon like face, central distribution of body fat, especially high on the back (buffalo hump), and fluid retention
  • Muscle cramps and weakness due to an overproduction of aldosterone, resulting in low body potassium
  • Increase in blood pressure
  • Short stature (growth failure)

How is Adrenocortical Carcinoma Diagnosed?

The diagnosis of Adrenocortical Carcinoma includes a through physical exam along with an evaluation of medical history and certain sophisticated diagnostic tests.

During the physical exam, the physician may look for:

  • Changes in the normal shape of the body
  • Symptoms of virilization (as described above)
  • Elevated blood pressure
  • Presence of an abdominal mass

Specific diagnostic tests may include:

  • Serum electrolytes, such as increased sodium and decreased potassium, due to excessive aldosterone production
  • To check the levels of hormones in blood that are secreted from the adrenal cortex, such as aldosterone, cortisol, and androgens
  • CT and MRI scan of the abdomen
  • Biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together the clinical findings, conducting special studies on tissues (if needed), and organizing the microscopic findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be the gold standard in arriving at a conclusive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Adrenocortical Carcinoma?

The complications of Adrenocortical Carcinoma may include:

The cancer can spread to other organs of the body, resulting in their involvement. These organs usually include:

  • Liver
  • Bone
  • Lung

A tumor invasion into a blood vessel can increase the risk of forming a thrombus (clot), which can suddenly travel to the brain, leading to death.

How is Adrenocortical Carcinoma Treated?

  • The primary treatment of Adrenocortical Carcinoma is a surgical resection (or removal) of the tumor
  • Radiation therapy and chemotherapy are other treatment options, depending upon the health status of the individual and spread of the tumor
  • Medications may be given to treat the related conditions of the tumor, such as reducing the excessive amount of cortisol production that causes a majority of the associated symptoms present with this tumor type
  • Post-operative care is important: A minimal activity is to be ensured, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Adrenocortical Carcinoma be Prevented?

  • Currently, there are no known or available protective measures to prevent the development of Adrenocortical Carcinoma.
  • Due to its metastasizing and recurrence potential, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor type

What is the Prognosis of Adrenocortical Carcinoma? (Outcomes/Resolutions)

  • Adrenocortical Carcinoma is a condition that generally has a poor prognosis.
  • However, there is a better prognosis, if the condition is detected early before the tumor spreads to other parts of the body (such as the liver, lung, and bone)
  • If the tumor has spread to other body parts and regions, then normally the survival period is estimated to be between 1-3 years

Additional and Relevant Useful Information for Adrenocortical Carcinoma:

Adrenocortical Carcinoma is a very rare condition that has been reportedly observed in about 1 person out of one million, every year, within the United States.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 17, 2015
Last updated: March 23, 2018