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Adrenal Disorders


Adrenal glands also called suprarenal glands, are small, triangular glands located on top of each kidney (one on each side) and measuring about one-half inch in height and 3 inches in length. The adrenal gland consists of two parts: the outer region called the adrenal cortex and the inner region called the adrenal medulla.

Function of the adrenal glands

The two parts of the adrenal glands, i.e. the adrenal cortex and the adrenal medulla perform separate functions. The adrenal cortex produces glucocorticoids (i.e., cortisol), mineralocorticoids (i.e. aldosterone) and androgens (DHEA and androstendione). Cortisol, a hormone essential for life has important effects on heart, blood pressure, immunity, metabolism, bone and nervous system. Both cortisol deficiency and overproduction will cause significant problems. Aldosterone is necessary for fluid and electrolyte (salt) balance and its excess may have clinical consequences. The adrenal androgen hormones cause problems mainly when overproduction occurs in women. The medulla is responsible for producing epinephrine and norepinephrine (adrenaline) with effects on blood pressure, heart and metabolism. Excess of medulla function will cause problems.

Regulation of the adrenal gland hormones

Cortisol is secreted by the adrenal glands in response to circadian and stress-induced stimulation by adrenocorticotropic hormone (ACTH) secreted by the pituitary gland. The pituitary gland is the "master" gland of the endocrine system, situated at the base of the brain. Aldosterone secretion is regulated by a system called renin-angiotensin and by potassium concentration, whereas pituitary gland is only a minor modulator.

In summary, the adrenal glands disorders consist of: adrenal insufficiency (deficiency in secretion, especially cortisol), adrenal hormone overproduction mainly caused by adrenal tumors (pheochromocytoma, aldosteronoma, androgen-producing tumors) and other adrenal tumors without hormone overproduction.

Adrenal Cancer and Metastases»

Adrenal cancer (primary adrenal carcinoma) is a rare, aggressive form of cancer in which cancer cells are found in the adrenal cortex, the outside layer of the adrenal gland.

Diagnosis of Adrenal Cancer

The symptoms are abdominal pain, weight loss and/or related to overproduction of hormones, such as cortisol and androgens (high blood pressure, high blood glucose, and increased hair and irregular periods in women).

Early diagnosis is vital, because the adrenal cancer may spread (metastasize) to other, distant organs.

Tests for adrenal cancer include:
  • Blood drawings
  • 24-hour urine collection
  • CT scan of the adrenal glands
Treatment of Adrenal Cancer

Treatment consists of surgery to remove the cancer whenever possible. There are medications available for situations when adrenal surgery is not an option, or in the case of recurrence, but they are not very effective.

Adrenal Incidentaloma»

Adrenal incidentaloma are masses or tumors (larger than one centimeter) that are found on the adrenal gland (the gland located above each kidney). The term “incidentaloma” refers to the fact that the mass is usually found “incidentally,” that is, while the patient is being examined for another condition, such as abdominal pain or injury. An adrenal incidentaloma is found in up to 5% of all people who are undergoing CT scans of the chest and abdomen, ultrasound of the abdomen, or magnetic resonance imaging (MRI) of the abdomen.

Diagnosis of Adrenal Incidentaloma

To make a diagnosis of adrenal incidentaloma, the physician will take the patient's history and conduct a physical examination. The patient will undergo a CT scan or an MRI. The physician will order blood and urine tests to rule out overproduction of hormones by the incidentaloma. Afterwards, the doctor will discuss the test results with the patient, and the doctor and the patient will decide on a treatment plan.

Treatment of Adrenal Incidentaloma

In deciding whether to treat a patient with an adrenal incidentaloma, the CT or MRI scan will indicate if the adrenal tumor is has malignant features, and the hormonal tests will indicate if the tumor is producing excessive adrenal hormones. The two options for treating adrenal incidentaloma are:

  1. Monitoring its appearance with a series of CT or MRI scans, or
  2. Removing the adrenal mass with surgery

If the first option is chosen, the doctor will inform the patient when and how often to come in for a scan.

Surgery is usually performed for cases of adrenal incidentaloma

  • if they look suspicious on the CT or MRI scan
  • the mass is very large and causing symptoms, or
  • the mass is secreting excessive adrenal hormones.

The surgery is usually performed by a surgeon who specializes in endocrine diseases (those that involve glands that secrete hormones) or a urologist (a physician who specializes in diseases of the urinary system).

Unless the adrenal incidentaloma is large, the surgery can be performed laparoscopically. Laparoscopic surgery uses a special camera called a laparoscope to produce an inside view of the body. Also called “keyhole” or “band-aid” surgery, laparoscopy is a minimally invasive procedure because it requires 3 or 4 small incisions rather than a single large one. The pencil-thin laparoscope and surgical instruments are inserted through the incisions.

Treatment consists of surgery to remove the cancer whenever possible. There are medications available for situations when adrenal surgery is not an option, or in the case of recurrence, but they are not very effective.

Adrenal Insufficiency (Addison’s disease)»

Addison’s disease, also known as adrenal insufficiency, occurs when underactive adrenal glands don’t produce enough of the hormone cortisol and, in some cases, the hormone aldosterone. The potentially life-threatening disease affects both sexes and can begin at any age. Approximately four in every 100,000 people have adrenal insufficiency.

In most cases, adrenal insufficiency results from the destruction of the adrenal cortex, the outer layer of the adrenal glands. This disorder is know as primary adrenal insufficiency and is usually an autoimmune condition in which a patient’s immune system uses its antibodies to attack and destroy its own adrenal glands. Some less common causes of primary adrenal insufficiency include: Tuberculosis, adrenal tumors and other infections.

Secondary adrenal insufficiency is a more common form of adrenal insufficiency and is due to a lack of adrenocorticotropin (ACTH). ACTH is a hormone secreted by the pituitary gland, which, in turn, stimulates the adrenal glands to produce cortisol, but not aldosterone. The lack of ACTH leads to a drop in the production of adrenal cortisol.

Abruptly ending treatment with glucocorticoids, such as prednisone is usually the most frequent cause of secondary adrenal insufficiency. Other causes of secondary adrenal insufficiency are less frequent and include: pituitary tumors, history of pituitary surgery, radiation

Symptoms of Adrenal Insufficiency
  • Weakness
  • Fatigue
  • Weight loss
  • Lack of appetite
  • Nausea
  • Vomiting
  • Abdominal pain
  • Constipation
  • Diarrhea
  • Darkening of the skin
  • Dizziness or lightheadedness upon standing
  • Craving salty foods
  • Muscle and joint pain

If the symptoms of adrenal insufficiency are left untreated, they may lead to severe nausea, vomiting, abdominal pain, and low blood pressure. Known as an adrenal crisis, its causes also include stressful events such as surgery, trauma, and infection. If an adrenal crisis is not treated, it can be fatal.

Diagnosis of Adrenal Insufficiency

Diagnosing adrenal insufficiency consists mainly of a series of blood tests to measure levels of cortisol in your system. If the levels are insufficient, CT and MRI scans may be used to determine whether the exact cause can be attributed to adrenal insufficiency.

To measure cortisol response, your doctor may order a stimulation test with either of the following:

Cortrosyn (ACTH) test

  • Usually done in the laboratory
  • Total duration of one hour

Insulin tolerance test

  • Done in Endocrinology outpatient department
  • Total duration of two hours

Once it has been determined whether the adrenal insufficiency is primary or secondary, your doctor may order an imaging (radiology) test for either your adrenal glands (usually a CT scan) or your pituitary gland (usually an MRI scan).

Treatment of Adrenal Insufficiency

Treatment of adrenal insufficiency involves replacing the insufficient hormones.

Treatment usually starts with either hydrocortisone or prednisone, corticosteroids that are used as cortisol replacements. The medication is taken orally once or twice a day. Adrenal treatment may also include taking fludrocortisone, a drug that replaces aldosterone, if needed. Patients should double the dose during stress, such as illness with fever, and let other health providers know about their condition prior to any procedures or surgeries. Patients who can’t take the oral medication due to nausea or vomiting should get medical attention and immediately receive injectable steroids to avoid adrenal crisis.

Since adrenal insufficiency is life-threatening, patients should wear a medical alert bracelet or necklace at all times.

Cushing’s Disease»

Cushing’s Disease occurs when a person is exposed for a long time to high levels of certain chemicals called adrenal glucocorticoids, one of which is called cortisol. Normally the adrenal glands (located on top of each kidney) make sufficient amount of cortisol which provides adequate response to stress. When there is too much cortisol in the body, symptoms of Cushing’s disease can occur.

Symptoms of Cushing's Disease
  • Weight gain and fat distribution mostly in the abdominal region
  • Easy bruising
  • Poor wound healing
  • Thinning of the skin (resulting in purple or pinkish stretch marks on many parts of the body)
  • Round face
  • Weak bones, which can lead to broken bones
  • Tiredness
  • High blood pressure
  • High blood sugar
  • Weak muscles
  • Irritability, anxiety, mood swings, and/or depression
  • Increased acne
  • Increased thirst and urination
  • Lack of menstrual periods; growth of facial hair (in women)
Causes of Cushing's Disease

In addition to the over-production of cortisol by the adrenal gland, symptoms of Cushing’s disease may occur if the person takes high dose steroids to treat other problems, such as rheumatoid arthritis, inflammatory bowel disease, lupus, and severe asthma. Steroids may also be given to patients who have had organ transplants. In some cases, a tumor on the pituitary gland, which stimulates the adrenal glands to produce cortisol, can lead to Cushing’s disease.

Frequency of Cushing's Disease

The type of Cushing’s disease that happens when the body makes too much cortisol is uncommon. Although this condition is rare, it can cause severe problems if it is not treated as soon as possible.

The type of Cushing’s disease caused by taking medicine is not uncommon because these medicines are so often used to treat many illnesses.

Cushing’s disease can occur in children and adults. When it develops in children and teens, it occurs equally in boys and girls. When it develops in adults, it affects women more than men.

Diagnosis of Cushing's Disease

There are some things your doctor can use to see if you have Cushing’s disease. These include:

  • Review of all the medicines you are now taking
  • 24-hour urine production
  • Blood tests
  • Radiographic tests such as MRI, or CT scan, and other more specialized tests to look for tumors on the adrenal or pituitary glands
Treatment of Cushing's Disease

Treatment varies on whether Cushing’s disease is caused by medicine or problems of the adrenal glands. If the symptoms are caused by high dose steroid treatment, the Endocrinologist may assist with the gradual dose adjustment if the doctor who first prescribed the medication agrees.

Treatment of adrenal or pituitary tumors that cause Cushing’s disease include:

  • Surgery
  • Radiation therapy
  • Use of drugs that reduce the production of cortisol

Pheochromocytoma»

Pheochromocytomas are tumors that develop in the adrenal medulla, the inner portion of the adrenal gland, which can cause overproduction of catecholamines. Catecholamines are hormones such as epinephrine and norepinephrine that are involved in maintaining blood pressure. Excess of catecholamines causes high blood pressure in most of the cases. Pheochromocytoma is a rare disease; of all patients with hypertension, less than one percent have adrenal tumors and the resulting hormone overproduction. However, if left untreated, it can have serious consequences, including heart attack, stroke, and other life-threatening conditions.

In most cases, a pheochromocytoma is a single, benign tumor that that occurs in a patient without a family history of endocrine problems. However, a small percentage may be linked to hereditary causes.

Symptoms of Pheochromocytoma

Typical symptoms may include high blood pressure and episodes of:

  • Sweating
  • Headache
  • Heart palpitations

These may be brought on by:

  • Emotional stress
  • Exercise
  • Anesthesia
  • Abdominal pressure
  • Indigestion due to tyramine-containing foods (e.g., some types of cheese)

Other symptoms include:

  • Lightheadedness upon standing
  • Weight loss
  • Shortness of breath
Diagnosis of Pheochromocytoma

When the doctor suspects pheochromocytoma, he or she will order blood tests and 24-hour urine collections. It’s important to abstain from alcohol and caffeine for at least 24 hours before the test, and stop some medications that may interfere with it as well. In some situations, the diagnosis may require additional testing.

Once the lab results are received, imaging (radiology) tests of the adrenal glands will be ordered. The three major tests used to locate a pheochromocytoma are the CT scan, MRI scan, the meta-iodobenzylguanidine (MIBG) scan. Your doctor will determine which scan is right for you.

Treatment of Pheochromocytoma

Whenever possible, the preferred treatment is to remove adrenal tumors surgically. This is usually done through laparoscopic surgery, which is minimally invasive and requires fewer days spent in the hospital after the procedure. However, your surgeon will decide which surgical method is appropriate for your situation. Medical treatment is indicated as a preparatory treatment prior to the adrenal surgery.

Primary Hyperaldosteronism (Conn’s Syndrome)»

Conn's Syndrome is overproduction of mineralocorticoids (aldosterone) by the adrenal glands.

Clinical presentation is very nonspecific and may include: high blood pressure, headaches, excessive urination and thirst, muscle weakness or cramps. Patients may be have no complaints but have hypertension that is refractory to conventional treatment or low potassium noticed on usual blood tests. The consequences of long standing hyperaldosteronism are related to high blood pressure, low potassium and include heart problems.

Diagnosis of Conn's Syndrome

A blood test and 24-hour urine collection is done, if necessary. It is extremely important that the patient reviews all his medications with the Endocrinologist before testing and follows the recommendation of stopping some of them if needed. Alternative medications for controlling high blood pressure will be prescribed if necessary. The patient might be asked to add one flat teaspoon of salt to their meal for five days and collect the urine during the last day of salt load. If laboratory tests confirm the diagnosis of primary hyperaldosteronism, radiological tests will be done next (usually CT scan of the adrenal glands).

Treatment of Conn's Syndrome

Laparoscopical adrenalectomy (removal of the adrenal gland that has the tumor).

Radiological tests for the adrenal glands

The CT Scan

The CT Scan (also called CAT scan) uses ionized radiation (x-rays) and is very accurate for the adrenal glands. The CT scan is painless and will take about 30 minutes to complete. The radiologist might decide to inject contrast material in a peripheral vein. If you recall an allergic reaction in the past to this material, you need to let the radiologist know before the injection.

The MRI Scan

The MRI Scan uses magnetic fields to generate pictures of body structures. The exam is painless and takes about an hour. Most MRI machines are closed. You should tell the radiologist if you had problems with claustrophobia during an MRI in the past. Also, the exam is contraindicated if you had metallic devices implanted in your body.

The MIBG Scan

The MIBG Scan is a nuclear medicine test used to detect the presence and location of adrenal pheochromocytomas. The radiologist will administer a radioactive dye which is a precursor for adrenaline (the hormone made by the adrenal medulla).

  • Bring a list of all medications along (or the medications)
  • If you ever had other tests regarding the adrenal problem (blood or urine tests, CT scans, MRIs) bring the reports. It would be great if you could obtain the film from Radiology and bring it along, as well.

Any other tests will be decided at the time of your visit. You are usually given written instructions regarding 24-hour urine collection and various tests to be performed.

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