What are the Adrenal Glands?
The body has two walnut-sized adrenal glands, one above each of the kidneys (‘ad-renal’ means ‘next to the kidney’). Even though the glands are small they are important as they produce several hormones (the body’s chemical messengers) that are important for life.
The adrenal medulla (the inner area of the adrenal gland) makes a number of hormones called catecholamines, mainly adrenaline and noradrenaline. These hormones help the body to maintain blood pressure and deal with sudden stress or threats.
The adrenal cortex (outer area of the gland) makes hormones called steroids, mainly cortisol (also known as glucocorticoid) and aldosterone (also known as mineralocorticoid). These steroids help the body to maintain blood pressure as well as salt and sugar levels. Cortisol is also an important messenger in our bodies’ response to stress and illness.
What is ACC?
There are several different types of tumors that can occur in the adrenal gland. They can develop in either the outer part of the gland (the cortex) or the inner part of the gland (the medulla). Tumors can be benign (not cancer) or malignant (cancer).
Benign tumors of the cortex are called adrenocortical adenomas, and malignant tumors are called adrenocortical carcinomas (ACC).
Adrenocortical carcinoma (ACC) is often known simply as adrenal cancer and affects 1-2 people per million per year, making it a rare form of cancer.
ACC in adults tends to occur in people in their 50s and 60s and is more common in women than in men. Most ACC’s are sporadic (meaning that they do not run in families), but they may sometimes be part of a congenital (present at birth) and/or familial (passed down in families) condition.
What are the symptoms of ACC?
Adrenocortical carcinomas (ACCs) are usually found because of the symptoms they cause but sometimes they are also found by chance if a patient has a scan for other reasons. ACCs may make higher than normal amounts of cortisol and aldosterone and may also secrete hormones that a healthy adrenal would not produce. These may include the male hormone testosterone and the female hormone estradiol that cause symptoms and even body changes. Some ACCs get very large and although these don’t usually make active hormones (they are non-functioning), they may cause symptoms by pressing on other organs in the body.
There are 2 types of ACC:
Hormone Secreting – This is when the tumor makes more than normal amounts of steroid hormones in addition to those already made by the normal adrenal gland cells.
High levels of steroid hormones cause symptoms that may include:
• high blood pressure
• sexual dysfunction,
• muscle weakness and wasting,
• weight gain,
• excess facial or body hair in women
• baldness in women
• deepening of the voice in women
• soreness and increase in the size of the breasts in men
• easy bruising,
• early puberty in children,
• reduced immunity (impaired response to infections),
• change in body shape
• mood changes.
Non-functioning – These tumors are likely to be found when symptoms occur due to the size of the tumor. Symptoms might include pain or swelling, weight loss or signs that the disease has spread outside of the adrenal gland. They do not cause the symptoms of high hormone levels, although sometimes these hormones are found in the blood or urine. Sometimes tumors are found by chance, such as during a scan while looking at something unrelated. These tumors are described as ‘incidental’.
Most adrenal nodules (small tumors) discovered by chance are harmless but larger tumors (usually more than 4cm) need careful checking for cancer.
What Causes ACC?
Scientists do not know exactly what causes most ACCs. However, over the past few years, they have made great progress in learning how certain changes in a person’s DNA can cause cells in the adrenal gland to become cancer. DNA carries the instructions for nearly all the things our cells do, and DNA changes can become risk factors for getting certain diseases including some types of cancer.
Most DNA changes that are seen in cancers happen during life rather than having been passed down in families. These changes may be caused by exposure to radiation, lifestyle or cancer-causing chemicals. But many of these changes happen for no known reason.
Most ACCs do not run in families; however, a number of rare conditions can increase the risk of developing ACC. Therefore, if a person with ACC has other members of the family who have been diagnosed with cancer aged 50 years or less, they should ask for a referral to their Regional Genetics Service.
How is ACC Found?
Very often, ACC tumors are not found until they have grown quite large. This is different for children since they react more to the hormones these tumors make. Sometimes, ACC may be found early by coincidence when having tests or scans for other unrelated health problems Usually, ACC is found due to the symptoms caused by either the hormones they make or because the tumor has grown large and is pressing on other organs in the body.
For more information visit the ACC Support UK website