The Adrenal Glands are responsible for many aspects of healthy bodily function. Sometimes, however, problems can arise with one or both glands. A problem with the adrenal glands is referred to as adrenal disease.
There are several kinds of adrenal disease that may require medical treatment. Malfunctioning glands adversely affect the body in a myriad of ways, and some can become life-threatening.
Below are some frequently asked questions and answers about adrenal disease.
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- GLANDS & HORMONES: BACKGROUND
- THE ADRENAL GLANDS
- ADRENAL HORMONES
- ADRENAL HEALTH
- ADRENAL DISEASES
- TREATING ADRENAL DISEASE
- ADRENAL SURGERY
What is the endocrine system?
In order for the body to carry out daily life, a complex system constantly occurs. Chemicals travel throughout the body like signal messengers to maintain healthy bodily functions. They communicate, coordinate, and regulate an enormous number of essential processes.
These chemical messengers are called hormones. The organs that produce the hormones are called glands. The endocrine system is the name for the full network of hormones and glands.
What are the Adrenal glands?
The adrenal glands are two small, triangular-shaped organs that sit atop both kidneys (in the back of the abdomen). Their function is to secrete a variety of hormones that are essential to maintaining bodily functions.
How do the Adrenal Glands affect the body?
The adrenal glands influence a wide array of bodily functions. These include:
- heart rate
- blood pressure
- stress responses
- immune response
- electrolyte balance
- food metabolism
- inflammation responses
- blood sugar management
- sex hormone production
- pregnancy health, and
- sexual development.
How many Adrenal Glands does a person have?
Most people are born with two adrenal glands.
To have normal function, people only need (part of) one adrenal gland. So there is a lot of reserve, by having two functioning adrenal glands.
Which hormones do the adrenal glands produce?
The adrenal glands secrete the hormones aldosterone, cortisol, and the sex hormones testosterone and estrogen.
They also create the “fight or flight” hormones, norepinephrine and epinephrine (better known as adrenaline).
How does one kind of gland produce so many different hormones?
Different parts of the adrenal gland create specific hormones. There is an outer portion of the adrenal gland and an inner portion. Respectively, these are the adrenal cortex and the medulla.
Which hormones are produced in the outer portion of the adrenal gland (adrenal cortex)?
The outer portion of the adrenal gland is called the adrenal cortex. It has three layers. Each layer makes a different hormone.
The outermost layer of the adrenal cortex produces the hormone Aldosterone, which controls blood pressure.
The middle layer of cortex makes cortisol. Cortisol controls the immune system, inflammation, and stress responses.
The innermost layer of the cortex produces the Sex hormones testosterone and/or estrogen. These control reproductive organs and development of male/female characteristics.
Which hormones are produced in the inner portion of the adrenal gland (adrenal medulla)?
The inner portion of the adrenal gland is called the adrenal medulla. It produces a group of hormones known as catecholamines: adrenaline (epinephrine) and noradrenaline (norepinephrine).
Catecholamines are hormones that increase the heart rate, blood pressure, rate of breathing and amount of energy available to the body.
Adrenaline is the most common and well-known catecholamine. The adrenal medulla releases extra adrenaline in response to stress. This increase is known as the "fight or flight response"—i.e. the body is ready to fight or run.
What is the “Fight or Flight” response?
In response to physical or emotional stressors the adrenal gland releases hormones that trigger various “survival instincts.” The phrase “fight or flight” refers generically to a person’s options, when potentially facing danger.
These “fight or flight” hormones send nerve impulses, via the brain, throughout the body. The result involves narrowing blood vessels, raising heart rate, and marshaling the body’s resources toward dealing with acute or perceived threats.
Part of that process also involves shoring up energy. For example, redirecting blood flow to major muscle groups might mean temporarily reducing the effort of the less immediately urgent immune system and digestive system.
What is healthy adrenal gland function? Is there a test?
Healthy adrenal glands produce and release hormones to meet the body’s requirements. That means that the balance and the amount of each hormone allow the body to operate normally. Adrenal hormones are not typically tested in otherwise healthy people.
The two things that will most commonly trigger adrenal hormone testing are (1) signs and symptoms of adrenal disease or (2) imaging findings.
Signs and symptoms would include things that point to hormone over- or underproduction. These include weight gain, proximal muscle weakness, easy bruising, high blood pressure diabetes for Cushing’s disease; high blood pressure, episodic palpitations and sweating for pheochromocytoma; and high blood pressure and low potassium for aldosteronoma.
If, during imaging testing for another condition, a mass were discovered in the adrenal, that would also trigger adrenal testing.
What are some possible Adrenal Gland disorders? What is Adrenal Disease?
Adrenal gland malfunction can occur in a few different ways. Specific conditions result from a variety of causes.
Both adrenal overproduction and adrenal failure are rare. Addison’s disease, which is when the adrenals aren’t working, has an estimated prevalence of 50 per million people in the general population.
What is Adrenal Cancer?
Adrenocortical carcinoma (ACC) is a rare but very dangerous cancer of the adrenal gland. ACC is found in 1 out of every million people and is most common either before 10 years old or between the ages of 50 and 60 years old.
Most adrenocortical carcinomas are not due to a genetic problem, although ACC can occur in families with two very rare genetic syndromes: Li-Fraumeni and Beckwith-Wiedemann Syndrome. These tumors usually grow and spread to other parts of the body rapidly, however, if caught early enough, ACC is potentially curable. Children tend to have a better prognosis because it is often found early, however since adults rarely have symptoms they are usually not found until the cancer is large and/or has spread to other parts of the body (i.e. metastasized).
In two-thirds of cases, tumors have already spread to surrounding organs or metastasized by the time of diagnosis, making patients Stage 3 or 4.
What is Addison’s Disease or Adrenal Insufficiency?
Addison's disease is a rare illness that occurs when the adrenal glands make too little of the hormones cortisol and aldosterone. Since it can be life-threatening, treatment involves taking hormone medications.
Symptoms can be slow-moving or acute, and include: salt-craving, extreme fatigue and weakness, cognitive and mood changes like irritability, depression, and delirium. Signs like darkened areas of skin, unintended weight loss, severe abdominal issues like vomiting or pain, muscle pain, and/or body hair loss can all be cause to see a doctor.
What is an Adrenal Incidentaloma?
An adrenal incidentaloma is an adrenal tumor that is discovered on an imaging test that is being done for a problem unrelated to adrenal disease. Adrenal tumors found as part of the work-up or follow-up of cancer are very likely to be adrenal metastases and do not count as adrenal incidentalomas.
As imaging techniques have improved and become more commonly used, doctors are finding more and more adrenal incidentalomas. The chance of having an adrenal incidentaloma increases with increasing age. At the age of 50, there is a 3% risk of having an adrenal incidentaloma and this goes up to 7% by the age of 70.
While most adrenal incidentalomas do not cause health problems, they must be evaluated because a small percentage can lead to significant disease.
Adrenal incidentalomas fall into one of three categories:
- Functioning tumors – these tumors make too much of any of the hormones that the adrenal glands normally make and include aldosterone-producing adenomas (See Primary Hyperaldosteronism), cortisol producing tumors (See Cushing's Syndrome), adrenaline producing tumors (See Pheochromocytoma), or sex-hormone producing tumors (See Sex-hormone Producing Tumors).
- Malignant tumors – these tumors include adrenocortical cancer and metastatic disease.
- Benign, non-functional tumors – these tumors include adenomas, myelolipomas, ganglioneuromas, adrenal cysts, and hematomas.
In general, tumors that are functional, malignant, and/or large should be removed.
What is Cushing's Syndrome?
Cushing's syndrome is a disease in which the body makes too much cortisol. Cortisol is a steroid that is made by the adrenal glands. Cortisol is necessary for daily life and helps the body respond to stressful events, regulates the sleep-wake cycles, and helps regulate the body's response to inflammation. A special part of the brain called the pituitary makes a hormone called adrenocorticotropic hormone (ACTH) that controls the release of cortisol from the adrenal glands. Although cortisol is essential to life, constantly high levels of cortisol can damage the entire body.
Hyperaldosteronism is a disease in which the adrenal gland(s) make too much aldosterone which leads to hypertension (high blood pressure) and low blood potassium levels. Aldosterone helps control blood pressure by holding onto salt and losing potassium from the blood. The increased salt increases the blood pressure.
Primary hyperaldosteronism can be caused by either hyperactivity in one adrenal gland (unilateral disease) or both (bilateral disease). Unilateral disease is usually caused by an aldosterone producing adenoma (benign tumor) and less commonly by adrenal cancer or hyperplasia (when the whole gland is hyperactive). Bilateral disease is usually caused by bilateral hyperplasia (when both glands are hyperactive). There are rare genetic syndromes like familial hyperaldosteronism type I and II which may cause both glands to be hyperactive.
Certain cancers can spread (metastasize) from other parts of the body to the adrenal gland, including kidney cancer (renal cell carcinoma), melanoma (a type of skin cancer), lung cancer, colon cancer, and lymphoma. The best treatment for metastatic cancer is usually systemic therapy like chemotherapy, however doctors will sometimes recommend removing the adrenal gland.
Adrenalectomy may be recommended when the primary disease is well controlled and the adrenal is the only site of metastatic disease, if the patient is having significant symptoms from a large adrenal tumor, or if a diagnosis needs to be made and the adrenal is the easiest site to perform a biopsy. It is uncommon for metastatic cancer to appear in the adrenal gland before the primary site is known.
What is Pheochromocytoma?
Pheochromocytomas are rare tumors that make too much adrenaline. Pheochromocytoma are found in 2 out of every million people each year and are the cause of high blood pressure in less than 0.2% of people with high blood pressure. However, because pheochromocytomas release adrenaline in uncontrolled bursts, they can cause serious health problems like stroke, heart attacks, and even death. For this reason, many doctors call it the "pharmacologic time bomb." Pheochromocytoma can occur at any age and there are no known risk factors except for certain genetic syndromes. They are often called the "10% tumor" because it was commonly thought that 10% of pheochromocytomas are:
- Malignant – 10% behave like cancer and can spread
- Bilateral – 10% are found in both adrenal glands
- Pediatric – 10% are found in children
- Extra-Adrenal – 10% are found outside the adrenal glands
- Familial – 10% are genetically caused.
However, as we learn more about this rare disease, we are discovering that some of these numbers may be higher. In fact, research shows that up to 25% of pheochromocytomas are inherited.
Even after removing the tumor it is often impossible to tell the difference between a malignant and benign pheochromocytoma under the microscope. The only definite way to tell if it is a malignant pheochromocytoma is if there is spread into nearby organs (i.e. local invasion), spread to distant sites like the lung and bone (i.e. metastases), or cancer that comes back (i.e. recurrence).
When pheochromocytomas are located outside the adrenal glands, they are called paragangliomas. (See Paraganglioma) Outside the adrenal glands, these tumors can be found anywhere along the sympathetic nervous chain. The most common extra-adrenal site is a point on the abdominal aorta (the largest artery in the abdomen) called the organ of Zuckerkandl.
What is Paraganglioma?
When pheochromocytomas are located outside the adrenal glands, they are called paragangliomas. Outside the adrenal glands, these tumors can be found anywhere along the sympathetic nervous chain. The most common extra-adrenal site is a point on the abdominal aorta (the largest artery in the abdomen) called the organ of Zuckerkandl.
What is a Sex-hormone Producing Tumor?
Sex-hormone producing adrenal tumors are rare tumors that make too much androgen (i.e. testosterone), estrogen, or both. These tumors are found in 2 out of every million people and can be either benign (adenomas) or malignant (adrenocortical cancer). Although smaller tumors are more likely to benign, while larger tumors are more likely to be malignant, the only way to determine if a sex-hormone producing tumor is cancer is if there is spread into nearby organs (i.e. local invasion), spread to distant sites like the lung and bone (i.e. metastases), or cancer that comes back (i.e. recurrence).
How are the adrenal gland and blood pressure related?
The adrenal gland makes two hormones that help with blood pressure. Aldosterone is one of those hormones. When blood pressure gets lower, aldosterone level goes up and that's how it helps regulate blood pressure. There's another hormone called cortisol that also has effects on the blood pressure. When your blood pressure goes down, your cortisol goes up.
If a person secretes too much of these hormones (especially aldosterone), their blood pressure can become too high. And, more rarely, if a person produces too little of these hormones, they can have low blood pressure.
People below the age of thirty with high blood pressure should be screened for hyperaldosteronism. So should people with resistant hypertension, meaning that despite multiple medications their blood pressure is still high.
Anyone with “very high” blood pressure, meaning greater than or equal to 180 over 120 or with high blood pressure that, all of the sudden, doesn't respond to medications that had previously controlled it well for years should think about being screened for hyperaldosteronism.
Finally, if a person’s potassium is consistently low and needs potassium supplementation to keep normal levels of the mineral should also be screened for hyperaldosteronism.
What kinds of doctors treat Adrenal Glands?
Endocrinologists are typically the first line in establishing a diagnosis of adrenal disease and setting up a treatment plan, and they help coordinate care with other adrenal specialists. Some endocrinologists specialize in one particular gland, such as the adrenal gland.
Surgery is often the most effective treatment for adrenal disease. Adrenal surgery is performed by surgeons trained in endocrine surgery.
Depending on the condition, additional input and care can come from oncologists, anesthesiologists, and a variety of specialists.
What tests are used to diagnose Adrenal Disease?
The most common test is for cortisol. The adrenal can either overproduce or underproduce cortisol. There is also a test for over- or underproduction of aldosterone.
There is a procedure called adrenal vein sampling to collect blood from each adrenal gland and compare the two sides to determine which side overproduction is coming from, or if it's from both. If the adrenal vein sampling determines that only one of the adrenal glands is responsible for the high level of aldosterone, surgery is an option. Otherwise, medication may be prescribed.
What is Non-Surgical Adrenal Treatment?
For Addison’s disease (adrenal insufficiency) the treatment is medication. For overproduction of cortisol, adrenaline, or other adrenal hormones, the treatment is usually surgery.
In situations where both adrenal glands are causing too much aldosterone, there are medications that can help with that.
Rather than surgically remove both glands, Spironolactone and Eplerenone are two medications that are used to antagonize or take up the receptor that aldosterone would bind to so that aldosterone can't bind there.
The aim is to help decrease blood pressure. And since the problem is with the aldosterone, the treatment is directed against the aldosterone, which can help people get their blood pressure and their potassium under control.
What is Adrenal Surgery?
The two main ways to perform an operation to remove the adrenal gland (i.e. adrenalectomy) are a minimally invasive or open operation. The most common minimally invasive operation is a laparoscopic adrenalectomy, which involves making 3 or 4 very small incisions and removing the adrenal gland using a small camera and special instruments.
Once a patient is advised to have an adrenalectomy, the decision regarding which approach to use is based on the features of the tumor including size, type, appearance on imaging studies, and whether or not the patient has had prior abdominal surgery. In general, the laparoscopic approach is favored over the open approach because laparoscopy significantly decreases discomfort, speeds healing, and leaves only tiny scars behind.
In addition, after laparoscopic adrenalectomy, the majority of patients are able to leave the hospital the day after the operation. At the Adrenal Center, we perform 98% of adrenal operations using minimally invasive techniques in order to provide patients with the least amount of disruption to their normal lives.
How long is hospitalization following adrenal surgery?
Adrenal surgery calls typically for one to two days of hospitalization. Most patients will be able to go home the day after surgery.
What type of anesthesia accompanies adrenal surgery?
Patients are completely asleep with general anesthesia for an adrenalectomy.
Does adrenal surgery leave a scar?
Yes, for the laparoscopic approach, most patients have three to four small incisions that are less than an inch in size. If a patient requires an open surgery, there will be a larger single incision.
Does pain follow an adrenal operation?
Most patients who have the laparoscopic surgery will have some discomfort that is well controlled with over the counter medication
Will adrenal surgery require stitches?
Yes, but the stitches are all internal so nothing needs to be removed at a later date. The incisions will be covered with skin glue that will peel off on it’s own after a week or so.
Are there any physical restrictions after adrenal surgery?
The main restrictions include no heavy lifting (greater than thirty pounds) for three weeks and no soaking in a pool or a bathtub for one week. Patients may shower the next day and engage in light exercise as soon as they feel up for it.
What are the potential complications unique to adrenal surgery?
The potential complications include bleeding or infection, which can occur with any surgery, however the risks are very low (a less than one per cent chance).
Injury to or removal of surrounding organs during adrenalectomy is a risk of less than one per cent.
The chance of needing to make a larger incision if the patient cannot tolerate the laparoscopic approach is also very rare (a less than two per cent risk).
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