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
Causes
The cause of adrenocorticol 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 10 years old
• Adults over 50 years old
• A genetic defect may cause adrenocortical cancer in some children, but the majority are non-hereditary
• Unhealthy lifestyle choices such as smoking and poor diet are risk factors for most cancers; some researchers suspect they may contribute to adrenocortical cancers as well
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:
• Hypercortisolism, also known as Cushing’s syndrome (30% of cases)–excess cortisol, the hormone which helps the body respond to stressful situations and infections
• Hyperaldosteronism, also known as Conn’s syndrome (2% of cases)–excess aldosterone, the hormone which helps the body maintain normal levels of sodium and potassium
• In women with functional tumors that release males hormones or virilization (20% of cases), 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
• Urine Tests
• 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
• Other 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. Your surgeon may remove the infected adrenal gland or glands in a surgery known as adrenalectomy . He or she will also need to remove any surrounding tissues or lymph nodes that have been infected by the cancer.
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.
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. 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.
