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Overview of Endocrine Neoplasia and Cancer | Endocrinology

What are endocrine neoplasia and cancer?

Endocrine neoplasia refers to growths or tumors that affect the hormone-producing endocrine system. Tumors develop in the adrenal glands, pituitary gland, parathyroid glands, or pancreas and can be cancerous or benign. The Nig Comprehensive Cancer Center‌ Endocrine Neoplasia Program is dedicated to the evaluation and treatment of the structural and hormonal symptoms of these disorders.

Our endocrine neoplasia program is the only one in Connecticut, and our dedicated team of experienced multidisciplinary specialists provides clinical services in the following areas:

  • Thyroid cancer and thyroid nodules
  • Fine injection aspiration biopsy: Thyroid and adrenal
  • Thyroid ultrasound
  • Thyroid carcinoma test: Thyrogen stimulated thyroglobulin and thyroid scan
  • Cancer and benign tumors of the adrenal glands.
  • Invasive radiology including petrous sinus sampling, adrenal vein sampling, and selective infusion of pancreatic calcium
  • Hyperparathyroidism and parathyroid tumors
  • Pituitary adenomas
  • Dynamic endocrine examination
  • Nuclear medicine scan

With specialists in a variety of diagnostic and therapeutic approaches, we work with teams of physicians from other disciplines to treat patients with endocrine neoplasia. Our medical professionals have experience in endocrine surgery, urological surgery, neurosurgery, neuro-ophthalmology, pathology, nuclear medicine, invasive radiology, hypertension, genetics, molecular biology, and endocrinology. They provide state-of-the-art patient care while conducting basic and clinical research to advance treatment options.

Types of endocrine neoplasia and cancer

Tumors can appear in any large endocrine gland, including the thyroid, parathyroid, pituitary, and adrenal glands, and the pancreas. The most common sites are:

  • Thyroid gland: Most endocrine cancers develop in the thyroid gland (a butterfly-shaped organ in the lower neck). Thyroid cancer is more common in women than in men. Statistics show that the annual rate of thyroid cancer is increasing in the United States and around the world. The good news is that most thyroid tumors (called nodules) are not cancerous.
  • Pituitary gland: A pea-sized organ connected to the brain, the pituitary gland produces hormones that affect growth and fertility. Pituitary tumors are almost always benign, but they contain more or less than one or more hormones, which can upset the balance of other glands.
  • Adrenal gland: The two adrenal glands that live above the kidneys produce hormones that regulate metabolism (cortisol), stress response (adrenaline), blood pressure (aldosterone), and certain sexual characteristics (androgens).
  • Pancreas: Although the pancreas plays an active role in the digestive system, it is also a source of important hormones, including insulin. Rare tumors produce too much insulin or other related hormones, which can affect blood sugar levels.

Although some cases are inherited, the cause of most endocrine cancers is generally unclear.

Symptoms of endocrine neoplasia and cancer

Some patients with thyroid tumors notice a lump in the neck. For others, and for other endocrine tumors, the general rules do not apply. Some tumors cause severe hormonal changes or discomfort, while other tumors do not have any symptoms.

So when does a tumor have symptoms? You basically have symptoms if it doesn’t work (makes extra hormones) but is active (doesn’t make them). For example, an adrenal tumor that produces excess testosterone can cause a patient to develop certain male characteristics, such as facial hair. Symptoms also appear as the tumor grows.

A large tumor destroys part of the gland, causing a lack of hormones. It also affects nearby structures. For example, a large pituitary tumor can focus on the nerve that runs between the eyes and the brain, causing vision changes. When endocrine tumors have no symptoms, doctors may randomly notice them and evaluate the patient for another reason.

What are the genes associated with multiple endocrine neoplasias?

Mutations in the MEN1, RET, and CDKN1B genes cause multiple endocrine neoplasms. Mutations in the MEN1 gene cause type 1 multiple endocrine neoplasias. This gene provides instructions for the production of a protein called melanin. Menin acts as a tumor suppressor, which means that it generally prevents cells from growing and dividing too quickly or uncontrollably.

Although the exact function of the meninges is unknown, it is involved in cellular functions such as DNA copying and repair and regulation of the activity of other genes. When mutations inactivate two copies of the MEN1 gene, the meninges are no longer available to control cell growth and division. Loss of functional meninges allows cells to divide more frequently, leading to tumor characterization of multiple endocrine neoplasia type 1. It is not clear why these tumors affect endocrine tissues.

Mutations in the RET gene can cause type 2 multiple endocrine neoplasias. This gene provides instructions for the production of a protein involved in cell signaling. The RET protein stimulates chemical reactions that direct cells to respond to their environment, for example by dividing or maturing. Mutations in the RET gene over-activate the protein’s signaling function, which stimulates cell growth and division in the absence of signals external to the cell. This unproven cell division can lead to the formation of tumors in the endocrine glands and other tissues.

Mutations in the CDKN1B gene cause type 4 multiple endocrine neoplasias. This gene provides instructions for the production of a protein called p27. Like the meaning protein, p27 is a tumor suppressor that helps regulate cell growth and division. Mutations in the CDKN1B gene reduce the number of functional p27 that allows cells to grow and divide without being analyzed. This irregular cell division leads to the development of tumors in the endocrine glands and other tissues.

Diagnosis of endocrine neoplasia and cancer

Doctors can perform several tests to check for a suspected endocrine tumor:

  • A medical history and physical exam to assess for physical or behavioral changes related to hormone function
  • Lab tests to check for abnormal hormone levels in the blood or urine
  • Imaging studies (CT scan, MRI, or ultrasound) to look for evidence of abnormal tissue in the gland
  • A biopsy to obtain a sample of abnormal tissue and analyze it for cancer cells

Treatment for endocrine neoplasia and cancer

For any endocrine tumor, treatment involves surgery to remove it. For people with cancer, another approach that is sometimes used is radiation therapy. Patients sometimes receive hormone therapy to balance the level of hormones in the body. Depending on the type of tumor, your doctor may prescribe other specific rules to meet your individual needs.

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Types of Adrenal Disease and Health | Endocrinology

What are adrenal disease and health?

Adrenal disease and health, also known as adrenal insufficiency, is an abnormal disorder that occurs when your body does not make enough hormones. In Addison’s disease, the adrenal glands above the kidneys produce very little cortisol and often very little aldosterone.

The adrenal disease occurs in all ages and in both sexes and can be fatal. Treatment consists of taking hormones to replace the missing ones. The adrenal gland consists of two parts: the outer part is called the adrenal cortex and the inner part is called the adrenal medulla.

The function of the adrenal glands

The two parts of the adrenal glands, namely the adrenal cortex and the adrenal medulla, perform different functions. The adrenal cortex produces glucocorticoids (ie, cortisol), mineral corticosteroids (aldosterone), and androgens (DHEA and androstenedione). The hormone cortisol, which is essential for life, has important effects on the heart, blood pressure, immune system, metabolism, bones, and nervous system.

Both cortisol deficiency and overproduction can cause major problems. Aldosterone is necessary for fluid and electrolyte (salt) balance and has additional clinical consequences. Adrenal androgen hormones mainly cause problems when overproduction occurs in women. The medulla is responsible for producing epinephrine and norepinephrine (adrenaline) with effects on blood pressure, heart, and metabolism. The problems are due to excessive spinal function.

Regulation of hormones of the adrenal glands

Cortisol is secreted by the adrenal glands in response to circadian and stress-induced stimuli by adrenocorticotropic hormone (ACTH) secreted by the pituitary gland. The pituitary gland is the “master” gland of the endocrine system at the base of the brain. Aldosterone secretion is regulated by a system called renin-angiotensin and by potassium, but the pituitary gland is only a small modulator.

In summary, adrenal gland disorders, adrenal insufficiency (secretion deficiency, especially cortisol), overproduction of adrenal hormones mainly adrenal tumors (pheochromocytoma, aldosteronism, androgen-producing tumors), and other adrenal glands that do not produce overproduction of hormones. Adrenal cancer and metastasis

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

Types of adrenal gland disorders

There are many types of adrenal gland disorders, which are the following:

Adrenal gland tumors

Most adrenal gland tumors (abnormal growths in the adrenal glands) are not cancerous. They often do not cause symptoms or require treatment. However, adrenal gland tumors can produce a wide variety of hormones, and hormone levels can be very high.

Carcinoma of the adrenal cortex

It is a cancerous adrenal tumor that develops in the outer layer of the adrenal gland. Cancerous adrenal tumors usually appear a few years after they begin to grow, during which time cancer has spread to other organs.

Cushing’s syndrome

Cushing’s syndrome is a rare disease caused by an excess of the hormone cortisol in the body. In some cases, Cushing’s syndrome develops from the chronic use of steroid drugs (drugs that act like cortisol in the body). In other cases, the body also makes more cortisol. This overproduction occurs for several reasons, including the presence of tumors (abnormal growth):

  • Pituitary gland tumor
  • Adrenal gland tumor
  • Tumor in another part of the body (these are called “ectopic” tumors and are most often found in the pancreas, lungs, or thyroid gland).

Congenital adrenal hyperplasia (CAH)

CAH is a common genetic disorder in which the body makes too little cortisol. People with CAH can also have other hormonal imbalances. For example, their bodies may not make enough aldosterone (which regulates blood pressure and levels of body salt and potassium), but they can make more androgens (which promote the development of male sex organs).

Pituitary gland

The pituitary gland is located at the base of the brain. Releases hormones that affect many functions of the body. Among these hormones is the adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to release the hormone cortisol.

Sometimes benign (non-cancerous) pituitary tumors or, very rarely, cancerous tumors, can grow in the pituitary gland and cause a variety of complications. Some pituitary tumors release more ACTH, which causes the adrenal glands to make more cortisol. Cushing’s disease refers to the pituitary tumors that cause Cushing’s syndrome.

Pheochromocytoma

Pheochromocytomas are part of a family of large tumors called paragangliomas. Pheochromocytoma is a tumor that develops in the adrenal medulla, the inner part of the adrenal gland. It produces adrenaline, which causes the body to overproduce this hormone. In most cases, the tumors are not cancerous and do not spread to other parts of the body. But in about 10% of cases, the tumors become cancerous and spread.

Adrenal gland suppression

When people take steroid drugs (drugs that act like cortisol in the body) such as prednisone, hydrocortisone, or dexamethasone they can suppress or reduce the normal activity of the adrenal glands. Steroid medications, usually prednisone, are prescribed to treat certain types of arthritis, severe allergic reactions, asthma, autoimmune diseases, and other conditions.

In general, anyone taking steroids will gradually take lower and lower doses, over time until they stop taking the drug completely. This is called a “cap” dose. When steroid medications are stopped abruptly, especially after several weeks or more, the adrenal glands may not be able to produce adequate amounts of steroid hormones (especially cortisol) for weeks or months. This condition can cause health problems due to an imbalance in hormone levels until the adrenal glands begin to function.

Symptoms of adrenal diseases

Some common symptoms of adrenal diseases are:

  • Fear
  • Chronic fatigue
  • Digestive problems
  • Aches and pains
  • Mild headache
  • Low blood pressure
  • Weight Loss
  • Hair loss

Adrenal disease is also associated with adrenal insufficiency. Symptoms of adrenal insufficiency:

  • Chronic fatigue
  • Lack of appetite
  • Abdominal pain
  • Muscular weakness
  • Unexplained weight loss

In more severe cases of adrenal disease, you may experience:

  • Disappointment
  • Nausea
  • Vomiting
  • Diarrhea
  • Low blood pressure
  • Hyperpigmentation
  • Foods to avoid

If you decide to try a healthy adrenal gland diet, doctors recommend limiting foods and beverages high in refined and processed sugar and unhealthy fats while maintaining blood sugar.

Some foods to avoid:

  • White sugar
  • White flour
  • Alcohol
  • Caffeine
  • soda
  • Fried food
  • Processed foods
  • Fast food
  • Artificial sweeteners
  • Mealtime is also important. Helps control blood sugar and adrenal glands.
  • It helps to eat breakfast and eat regularly throughout the day. Skipping breakfast and lunch will force your body to burn stored nutrients and lower your energy levels.
  • By eating balanced meals and healthy snacks on a regular basis, you can maintain your energy and cortisol levels throughout the day.
  • Food to eat
  • A well-balanced diet is the best way to keep your body healthy and regulate your sugar levels. Doctors recommend balancing protein, healthy fats, and high-quality, nutrient-rich carbohydrates.
  • Increase your vegetable intake to get the necessary amount of vitamins and minerals. Also, foods high in vitamin C, vitamin B (especially B-5 and B-6), and magnesium can help keep the adrenal glands healthy.

Some foods to eat on the adrenal fat diet:

  • Fine meats
  • Fish
  • Eggs
  • Vegetables
  • Walnuts
  • Vegetables and collard greens
  • Cereals
  • Milk
  • Low sugar fruits
  • Sea salt is moderate
  • Healthy fats like olive oil, coconut oil, and grapeseed oil
  • Boiling is also important. Dehydration affects your stress levels and forces your adrenal glands to produce cortisol.

Causes of adrenal diseases

Damage to the adrenal glands causes adrenal disease, which results in a deficiency of the hormone cortisol, and often not enough aldosterone. Your adrenal glands are part of your endocrine system. They produce hormones that give instructions to virtually every organ and tissue in your body.

Your adrenal glands are made up of two sections. The interior (medulla) produces hormones such as adrenaline. The outer layer (cortex) produces a group of hormones called corticosteroids. Corticosteroids:

Glucocorticoids: These hormones, which contain cortisol, affect your body’s ability to convert food into energy, play a role in your immune system’s inflammatory response, and help your body respond to stress.

Mineral corticosteroids: These hormones, which contain aldosterone, help maintain your body’s sodium and potassium balance to maintain normal blood pressure.

Androgens: These male sex hormones are produced in small amounts by the adrenal glands in both men and women. They cause sexual development in men and affect muscle mass, sex drive (libido), and feelings of well-being in both men and women.

Primary adrenal insufficiency: When the cortex is damaged and does not produce enough adrenocortical hormones, this condition is called primary adrenal insufficiency. It is the result of an attack on the body (an autoimmune disease). For unknown reasons, your immune system sees the adrenal cortex as something foreign, something to attack and destroy. People with Addison’s disease are more likely to have another autoimmune disease than others.

Other causes of the adrenal disease include:

  • Tuberculosis
  • Other adrenal gland infections
  • Spread of cancer to the adrenal glands
  • Bleeding in the adrenal glands, In this case, you may have an Addisonian crisis with no previous symptoms.

Secondary adrenal insufficiency: The pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH). ACTH stimulates the adrenal cortex to produce its hormones. Benign pituitary tumors, inflammation, and pre-pituitary surgery are common causes of insufficient production of pituitary hormones.

Even if your adrenal glands are not damaged, too little ACTH leads to the glucocorticoids and androgens that your adrenal glands normally produce. This is called secondary adrenal insufficiency. The production of mineral corticosteroids is not affected by very low levels of ACTH.

Many of the symptoms of secondary adrenal insufficiency are similar to the symptoms of primary adrenal insufficiency. However, people with secondary adrenal insufficiency do not have hyperpigmentation and are less likely to develop severe dehydration or low blood pressure. They are more likely to have low blood sugar levels.

People taking corticosteroids (eg, Prednisone) to treat chronic conditions such as asthma or arthritis may have a temporary cause of secondary adrenal insufficiency when they stop taking corticosteroids all at once instead of using the tape.

Diagnosis of adrenal diseases

Your doctor will first talk to you about your medical history and your signs and symptoms. You will be subjected to some of the following tests:

Blood test: The tests can measure your blood levels of sodium, potassium, cortisol, and adrenocorticotropic hormone (ACTH), which stimulate the adrenal cortex to produce its hormones. A blood test can also measure the antibodies associated with autoimmune Addison’s disease.

ACTH stimulation test: ACTH refers to the adrenal glands producing cortisol. This test measures the level of cortisol in your blood before and after a synthetic ACTH injection.

Insulin-induced hypoglycemia test: Doctors may do this test if they think you have adrenal insufficiency as a result of pituitary disease (secondary adrenal insufficiency). The test will check your blood sugar (blood glucose) and cortisol levels after taking insulin. In healthy individuals, glucose levels drop and cortisol levels rise.

In some cases, doctors may perform alternative tests for secondary adrenal insufficiencies, such as a low-dose ACTH stimulation test, a chronic ACTH stimulation test, or a glucagon stimulation test.

Imaging tests: A computed tomography (CT) scan of your abdomen may be done to check the size of your adrenal glands and look for other abnormalities. You may have an MRI of the pituitary gland if the test indicates that you have a secondary adrenal defect.

Treatment of adrenal diseases

All treatment for adrenal disease includes medication. Hormone replacement therapy will be given to correct the levels of steroid hormones that your body does not make. Some treatment options include oral corticosteroids:

  • Cortisol is replaced by hydrocortisone (Cortef), prednisone, or methylprednisolone. These hormones are given on a schedule to mimic the normal 24-hour fluctuations in cortisol levels.
  • Fluidrocortisone acetate instead of aldosterone.
  • You should get a lot of salt (sodium) in your diet, especially when you’re exercising a lot when it’s hot, or if you have gastrointestinal problems like diarrhea.
  • Your doctor may also prescribe a temporary increase in your dose of action if your body is stressed, such as during an operation, infection, or minor illness. If you are vomiting and cannot reduce oral reactions, you may need corticosteroid injections.

Other treatment recommendations for the adrenal disease include:

  • Carry a medical alert card and bracelet with you at all times. The Steroid Emergency Card and Medical Alert ID will tell emergency medical personnel what kind of care they need. Also, include a written action plan.
  • Keep extra medications easy. It is dangerous to miss even one day of medication, so carry a small amount of medication with you when you travel.
  • Bring a glucocorticoid injection kit. The injection form of the needle, the syringe, and the corticosteroids in the kit are used in emergency cases.
  • Stay in touch with your doctor. Maintain an ongoing relationship with your doctor to make sure the dose of hormone replacement hormones is adequate, but not excessive. If you have ongoing problems with your medication, you may need to adjust your medication dose or schedule.
  • Carry out annual checks. See your doctor or endocrinologist once a year. Your doctor may recommend annual checkups for many autoimmune diseases.

Treatment for an Addison crisis, which is a medical emergency, usually involves intravenous injections:

  • Corticosteroids
  • Saltwater
  • Sugar (dextrose)
  • Future treatments

Researchers are working to develop a late-release corticosteroid that works like a human body. They also work with pumps placed under the skin, which can deliver steroids in more precise doses.

The use of adrenocortical stem cells in combination with immunomodulatory therapy at the end of future treatment, modifying the immune response of the immune system, as well as gene therapy.

 

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Prolactinoma | Complications and Treatment Options | Endocrinology

What is prolactinoma?

Prolactinoma is a non-cancerous tumor of the pituitary gland. This tumor overproduces a hormone called pituitary prolactin. The main effect of prolactinoma is a decrease in the levels of certain sex hormones: estrogen in women and testosterone in men.

Although prolactinoma is not fatal, it can cause vision problems, infertility, and other problems. Prolactinoma is a hormone-producing tumor in the pituitary gland. Sometimes the tumor grows in the pituitary gland and makes more prolactin. This type of tumor is called a prolactinoma. It is the most common type of pituitary tumor and is most often benign, which means it is not cancerous.

The new mother’s prolactin levels increase each time her baby breastfeeds. But for men or women who are not breastfeeding, high prolactin in the blood is a sign of prolactinoma. Doctors do not know what causes this, but it is very common. It occurs more often in women than in men and is very rare in children. They can often treat prolactinoma with medicine to bring your prolactin level back to normal. Surgery to remove the pituitary tumor is also an option.

Why is a prolactin levels test done?

Woman

Women with prolactinoma symptoms may need testing. Prolactinoma is a non-cancerous tumor in the pituitary gland that produces high levels of prolactin.

Symptoms of prolactinoma in women:

  • Unexplained headache
  • Vision error
  • Galactorrhea, or lactation outside of childbirth or lactation
  • Pain or discomfort during sexual intercourse.
  • Abnormal growth of body and facial hair.
  • Abnormal pimples
  • People with prolactinoma usually have tests to find out how the tumor responds to treatment.

Also, you may need a prolactin test if you have fertility problems or irregular periods. The test can also rule out other problems with the pituitary gland or hypothalamus.

Men

Men need testing if they have symptoms of prolactinoma. Symptoms of prolactinoma in men:

  • Unexplained headache
  • Vision error
  • Decreased sex drive or fertility problems.
  • Erection
  • Abnormal lack of body and facial hair

The test can also use:

  • Investigate testicular dysfunction or erection
  • Eliminate problems with the pituitary gland or hypothalamus

Symptoms of prolactinoma

The signs of prolactinoma are different in men and women. For women, the most common symptoms of a small tumor are:

  • Period of irregular structure
  • Lack of structure periods
  • Less sexual desire
  • Traumatic intercourse (caused by vaginal dryness)
  • Fertility problems
  • Breast milk is an unusual product

Since changes in the female period are evident, prolactinomas are more likely to appear early in premenopausal women. Women who have stopped menstruating can no longer have their period and do not notice symptoms when the tumor is small. When the prolactinoma is large, it can press against other nearby tissues. Symptoms include vision loss, vision changes, and headaches. When a man is diagnosed with prolactinoma, it can be large. Men are often undiagnosed when there are early signs, including:

  • Problems getting or keeping an erection
  • Decreased interest in sex
  • In rare cases, the production of breast milk.
  • Headaches or vision problems are symptoms that men often see a doctor.

Causes of prolactinoma

Prolactinoma is a type of tumor that develops in the pituitary gland. The cause of these tumors is unknown.

The pituitary gland is a small, bean-shaped gland located at the base of your brain. Despite its small size, the pituitary gland affects almost every part of your body. Its hormones help regulate important functions such as growth, metabolism, blood pressure, and reproduction.

Other causes of prolactin overproduction include medications, other types of pituitary tumors, thyroid gland dysfunction, persistent irritation of the breast, pregnancy, and breastfeeding.

Diagnosis of prolactinoma

If you have signs and symptoms that indicate you have a prolactinoma, your doctor may recommend:

  • Blood test: Blood tests can detect high levels of prolactin and other hormone levels that are regulated by the pituitary. Women of childbearing age also take a pregnancy test.
  • Mental image: Your doctor will be able to detect a pituitary tumor in an MRI image of your brain.
  • Vision tests: These will determine if the pituitary tumor is affecting your vision.

Also, your doctor may refer you for a more comprehensive examination with a doctor who specializes in treating endocrine system disorders (endocrinologist).

Prolactinoma treatment

Goals in the treatment of prolactinoma:

  • Restore prolactin production to normal levels.
  • Restore normal function of the pituitary gland
  • Shrink the pituitary tumor
  • Eliminate any signs or symptoms of tumor pressure, such as headaches or vision problems.
  • Improve Life Quality

Treatment of prolactinoma involves two main therapies: Medication and surgery.

Drugs

Oral medications often reduce prolactin production and eliminate symptoms. Medicines can also shrink the tumor. However, long-term treatment with medications is usually necessary.

Doctors use drugs called dopamine agonists to treat prolactinoma. These drugs mimic the effects of dopamine, a brain chemical that normally regulates prolactin production. Commonly prescribed medications include bromocriptine (Cycloset, Parlodel) and cabergoline. These drugs reduce prolactin production and can shrink the tumor in most people with prolactinoma.

Common side effects

Common side effects of these medications are nausea and vomiting, nasal pain, headache, and drowsiness. However, your doctor can reduce these side effects by starting with a very small dose and gradually increasing the dose.

There have been rare cases of heart valve damage with cabergoline, but it is more common in people taking high doses of Parkinson’s disease. Some people can develop compulsive behaviors such as gambling while taking these medications.

If the drug significantly shrinks the tumor and your prolactin level is normal for two years, you can lower the drug under the guidance of your doctor. However, recurrence is common. Don’t stop taking your medications without your doctor’s permission.

Medications during pregnancy

Bromocriptine is often prescribed when treating women who want to restore fertility. However, after you get pregnant, your doctor may recommend that you stop taking the medicine.

Although both drugs are considered safe in early pregnancy, their safety during pregnancy is unknown. However, if you have a large prolactinoma or have signs and symptoms such as headaches or vision changes, your doctor may recommend that you resume your medication to prevent prolactinoma complications.

If you are being treated for this disease and want to start a family, it’s a good idea to discuss your options with your doctor before you get pregnant.

Surgery

Surgery to remove the tumor is usually an option if drug treatment for this disease doesn’t work or if you can’t tolerate the drug. Surgery may be needed to reduce pressure on the nerves that control your vision. The type of surgery you undergo depends largely on the size and extent of your tumor:

  • Nasal surgery: Most people who need surgery have a procedure to remove the tumor through the nasal cavity. This is called transsphenoidal surgery. Complication rates are lower because other areas of the brain are not touched during surgery and this surgery does not leave visible scars.
  • Transcranial surgery: If your tumor is large or has spread to nearby brain tissue, you may need this procedure, also known as a craniotomy. The surgeon removes the tumor through the top of the skull.

The surgical outcome depends on the size and location of the tumor and its prolactin levels before surgery, as well as the experience of the surgeon. The higher the prolactin level, the lower the chances that prolactin production will return to normal after surgery.

Surgery corrects the prolactin level in most people with small pituitary tumors. However, many pituitary tumors come back within five years of surgery. For people with larger tumors that can only be partially removed, drug therapy often can return the prolactin level to a normal range after surgery.

Radiation

For people who don’t respond to medication and aren’t candidates for surgery, radiation therapy may be an option.

Complications of prolactinoma

Complications of prolactinoma can include:

  • Bone loss (osteoporosis): Too much prolactin reduces the production of the hormones estrogen and testosterone, which causes a decrease in bone density and an increased risk of osteoporosis.
  • Pregnancy problems: During a normal pregnancy, estrogen production increases. If you are pregnant and have a large prolactinoma, these high estrogen levels can lead to tumor growth and signs and symptoms such as headaches and vision changes.
  • Sight loss: If left untreated, it can grow as large as the optic nerve. You lose your peripheral vision.
  • Low levels of other pituitary hormones: With large prolactinomas, stress on the normal pituitary gland leads to levels of other hormones that are regulated by the pituitary, including thyroid hormones and cortisol (stress-responsive hormone).
  • If you have this disease and want to or are already pregnant, talk to your doctor. Adjustments in your treatment and supervision may be necessary.
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Symptoms & Complications of Sheehan’s Syndrome | Endocrinology

What is Sheehan’s syndrome?

Sheehan’s syndrome is a condition that occurs when the pituitary gland is damaged during childbirth. It is caused by excessive blood loss (hemorrhage) or extremely low blood pressure during or after childbirth. Lack of blood deprives the pituitary of the oxygen it needs to function properly.

The pituitary gland is at the improper of the brain. It produces hormones that monitor the function of the other glands in your body. That is why it is identified as “the master gland”. This gland is more vulnerable to injury during labor because it increases in size during pregnancy.

When the pituitary doesn’t work as well as it should, the glands it controls, including the thyroid and adrenal glands, can’t release enough hormones. Sheehan’s syndrome affects the production of these pituitary hormones:

  • Thyroid-stimulating hormone (TSH) directs the thyroid gland to produce its hormones, which regulate its metabolism.
  • Luteinizing hormone (LH) helps regulate your menstrual cycle and egg production, along with FSH.
  • Follicle Stimulating Hormone (FSH) helps regulate your menstrual cycle and egg production, along with LH.
  • Growth hormone (GH) controls the growth of organs and tissues.
  • Adrenocorticotropic hormone (ACTH) stimulates the adrenal glands to release cortisol and other stress hormones.
  • Prolactin stimulates milk production.

Sheehan’s syndrome is also called postpartum hypopituitarism.

Symptoms of Sheehan’s syndrome

The pituitary gland produces hormones that stimulate breast milk production, growth, reproductive functions, the thyroid, and the adrenal glands. The lack of these hormones can cause a variety of symptoms. While some women may experience early symptoms, such as difficulty breastfeeding or the inability to breastfeed (breast milk never “comes in”), in most cases the signs and symptoms of Sheehan’s syndrome advance slowly, sometimes over a period of months or even years.

For many, symptoms become apparent when the body is stressed by infection or surgery sometime after delivery. This type of stressor can lead to an adrenal crisis. Symptoms that may develop include:

  • Fatigue
  • Pain in the joints
  • Hypoglycemia
  • dizziness
  • Amenorrhea (lack of menstrual bleeding)
  • Oligomenorrhea (infrequent menstrual periods)
  • Hot flushes
  • Decreased libido
  • Loss of pubic and armpit hair
  • Low blood pressure (hypotension)
  • Slow mental functioning
  • Weight gain
  • Difficulty staying warm
  • Arrhythmia
  • Contraction of the breasts

Causes of Sheehan’s syndrome

Sheehan’s syndrome is caused by severe blood loss or tremendously low blood pressure through or after delivery. These factors can be particularly harmful to the pituitary gland, which enlarges during pregnancy and destroys hormone-producing tissue so that the gland cannot function normally.

Pituitary hormones regulate the rest of your endocrine system, signalling other glands to increase or decrease the production of the hormones that control metabolism, fertility, blood pressure, breast milk production, and many other vital processes. Lack of any of these hormones can cause difficulties throughout the body.

The hormones from the front of your pituitary gland include:

  • Growth hormone (GH): This hormone pedals bone and tissue growth and maintains the proper stability of muscle and fat tissue.
  • Thyroid-stimulating hormone (TSH): This hormone stimulates the thyroid gland to produce key hormones that regulate your metabolism. The shortage of TSH results in an underactive thyroid gland (hypothyroidism).
  • Luteinizing hormone (LH): In women, LH regulates estrogen.
  • Follicle Stimulating Hormone (FSH): Working with LH, FSH helps to stimulate egg development and ovulation in women.
  • Adrenocorticotropic hormone (ACTH): This hormone stimulates the adrenal glands to produce cortisol and other hormones. Cortisol assistances your body deal with stress and influences many bodily functions, including blood pressure, heart function, and immune system.

A low level of adrenal hormones caused by damage to the pituitary gland is called a secondary adrenal deficiency.

  • Prolactin: This hormone adjusts the development of female breasts, as well as the production of breast milk.

Risk factors

The main risk factor for the development of Sheehan’s syndrome in pregnancy. The pathophysiological variations of the pituitary gland during pregnancy leave the gland weak to ischemia in the event of bleeding. Similar blood loss in non-pregnant women does not cause hypopituitarism, lending credence to this claim. Postpartum hemorrhage can be tough to predict due to the unpredictable nature of the delivery procedure.

It is known that women who have experienced a previous postpartum hemorrhage are three times more likely to have a postpartum hemorrhage in their subsequent pregnancies. A situation analysis concluded that those diagnosed with Sheehan’s syndrome have a significantly higher disseminated intravascular coagulation (DIC) score than their non-disease counterparts. The DIC score, which is based on the patient’s disease history, clinical symptomatology, and coagulation laboratories, may be an important determinant of the potential for disease development.

Women with Sheehan’s syndrome report more pregnancy complications than their counterparts who do not have it. The linked complications of stillbirth, miscarriage, abruption, placental retention, uterine rupture, hysterectomy, and multiple pregnancies are related to the potential for bleeding and are more frequently reported and known to cause potentially life-threatening bleeding risks.

Complications

Complications of Sheehan’s syndrome include:

  • Adrenal crisis, a life-threatening condition in which the adrenal glands do not make enough cortisol, the stress hormone
  • Low blood pressure
  • Unexpected weight loss
  • Irregular periods

Diagnosis of Sheehan’s syndrome

Diagnosing Sheehan’s syndrome can be difficult. Many symptoms overlap with those of other conditions. To diagnose Sheehan’s disease, your doctor will likely:

  • Collect a complete medical history: It is important to mention any complications you had during labour, no matter how long ago you gave birth. Also, be sure to tell your doctor if you didn’t produce breast milk or were unable to start your period after delivery, two key signs of Sheehan’s syndrome.
  • Take blood tests: Blood tests will check your levels of pituitary hormones.
  • Request a pituitary hormone stimulation test: You may need a pituitary hormone stimulation test, which involves injecting hormones and doing repeated blood tests to see how your pituitary responds. This test is usually done after consulting a doctor who specializes in hormonal disorders (endocrinologist).
  • Request imaging tests: You may also need imaging tests, such as an MRI or CT scan, to check the size of the pituitary gland and look for other possible reasons for your symptoms, such as a pituitary tumor.

Treatment of Sheehan’s syndrome

Treatment for Sheehan’s syndrome is a lifelong hormone replacement therapy for the hormones you are missing. Your specialist may mention one or more of the following medications:

  • Corticosteroids: Hydrocortisone (Cortef) or prednisone (Rayos) replaces adrenal hormones that are not produced due to a deficiency of adrenocorticotropic hormone (ACTH).

You will need to adjust your medication if you become seriously ill or experience significant physical stress. During these times, your body would normally make extra cortisol, a stress hormone. The same type of dose adjustment may be necessary when you have the flu, diarrhoea or vomiting, or are undergoing surgery or dental procedures.

Dose changes may also be necessary during pregnancy or with obvious weight gain or loss. Taking the right amount can help avoid the side effects associated with high doses of corticosteroids.

  • Levothyroxine (Levoxyl, Synthroid, others): This medicine increases deficient thyroid hormone levels caused by poor or deficient production of thyroid-stimulating hormone (TSH).

If you change brands, tell your doctor to make sure you keep getting the correct dose. Also, do not skip doses or stop taking the medicine because you feel better. If you do, the signs and symptoms will gradually return.

  • Estrogen: This includes estrogen-only if you have had your uterus removed (hysterectomy) or a combination of estrogen and progesterone if you still have a uterus.

Estrogen use has been linked to an increased risk of blood clots and strokes in women who still produce their own estrogen. The risk should be lower in women who are replacing the missing estrogen.

Preparations containing luteinizing hormone (LH) and follicle-stimulating hormone (FSH), also called gonadotropins, may make future pregnancies possible. These can be given by injection to stimulate ovulation.

After age 50, around the time of natural menopause, talk with your doctor about the risks and benefits of continuing to take estrogen or estrogen and progesterone.

  • Growth hormone: The growth hormone can improve the relationship between muscles and body fat, maintain bone mass, and lower cholesterol levels. The growth hormone is expensive and side effects can include stiff joints and fluid retention.