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Adrenal Cancer

What Is Adrenal Cancer?

Adrenal cancer is a rare disease that originates in the adrenal glands (also known as Adrenal Cortical Cancer). The adrenal glands are located on top of the kidneys and consist of two parts that function separately: the outer layer (cortex) and the inner area (medulla). The cortex produces three major hormones: cortisol (a glucocorticoid), aldosterone (a mineralocorticoid), and dehydroepiandrosterone (DHEA; an androgen). The medulla produces epinephrine (adrenaline), norepinephrine, and dopamine.
Adrenal tumors can increase hormone production. Adrenal tumors that do not produce hormones are called nonfunctioning. Symptoms of adrenal cancer and treatment for the condition depend on whether the tumor is functioning or nonfunctioning, and on which hormone is being overproduced.

What Are the Risk Factors for Adrenal Cancer?

A family history: Although the vast majority of adrenal cortex cancers are sporadic (not related to heredity), some, particularly in children, are caused by a genetic defect. The Li-Fraumeni syndrome (a syndrome is a group of signs and symptoms caused by a particular condition) is a very rare condition in which a genetic defect leads to a greatly increased risk of developing several types of cancers.

Multiple endocrine neoplasia (MEN-1) is a condition that is inherited by about half of the children of a parent with the condition. People with MEN-1 have a very high risk of developing tumors of three glands — the pituitary, parathyroid, and pancreas. About one third of people with this condition also develop adrenal cortical adenomas, which tend to be small and usually do not cause any symptoms. Adrenal cortical cancers do not commonly occur in people with MEN-1.

Age: Many types of cancer are most common in older people. Adrenal cortical carcinomas, however, are most common among adults around ages 40-50 and among children.
Lifestyle and environmental factors: Risk factors such as a high-fat diet, smoking, sedentary lifestyle, and exposure to cancer-causing substances in the environment have a great impact on a person’s risk of developing many types of cancer.
Symptoms of adrenal cancer

About two out of three adrenal cortical cancers make hormones. If you have one that does not, you may not have any symptoms until the cancer is quite far advanced. Then you may start to have pain, tiredness and weight loss.

If your tumour makes hormones, these will cause your symptoms. If your tumour makes too much cortisol or aldosterone, you may have

  • Raised blood pressure
  • Thirst
  • Passing urine frequently
  • Muscle cramps
  • Weight gain that is more noticeable in the trunk of the body
  • Muscle wasting in the legs or arms, making them look thinner
  • Fat accumulation in the base of the neck (known as buffalo hump)
  • Swelling of the face giving a ‘moon face’ appearance
  • Mild diabetes (less common)
  • Abnormal hair growth on the face, arms and upper back

Do bear in mind there are many other causes for raised blood pressure which are far more common than adrenal gland cancer.

If your tumour overproduces sex hormones

  • Women may have deepening of the voice, changes in periods, baldness or growth of facial hair
  • Men may have loss of sex drive (libido), impotence and sometimes swelling of the breasts

Treatment

Treatment is aimed at removing the tumor by surgery. In some cases, this can be done by laparoscopy. Surgery is sometimes followed by chemotherapy and/or radiation therapy. Because the surgery removes the source of many important hormones, hormones must be supplemented following surgery. If adrenocortical cancer recurs or has spread to other parts of the body (metastasized), additional surgery may be done followed by chemotherapy using the drug mitotane.

Adrenal cancer sites

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