Panhypopituitarism

What is panhypopituitarism?

Panhypopituitarism is a rare condition in which there’s a lack (deficiency) of all of the hormones your pituitary gland makes. It can affect infants, children and adults.

Hormones are chemicals that coordinate different functions in your body by carrying messages through your blood to your organs, muscles and other tissues. These signals tell your body what to do and when to do it.

Your pituitary gland is a pea-sized gland located at the base of your brain below your hypothalamus (the part of your brain that controls your autonomic nervous system). It’s a part of your endocrine system.

The pituitary hormones are in charge of several important functions in your body, such as metabolism, growth and reproduction.

Normally, your body carefully controls hormone levels. If the levels of any of these hormones are unbalanced, it causes symptoms and health issues. Panhypopituitarism causes several symptoms since all of your pituitary hormone levels are lower than what they should be.

What is the difference between panhypopituitarism and hypopituitarism?

Panhypopituitarism is a type of hypopituitarism.

If you have a deficiency (lack) of one or multiple hormones your pituitary gland makes, you have hypopituitarism.

Panhypopituitarism happens when there’s a deficiency in all of the hormones your pituitary makes. The prefix “pan-” means “all.”

What hormones does the pituitary gland make?

Your pituitary gland makes and releases the following hormones:

• Adrenocorticotropic hormone (ACTH or corticotropin): This hormone stimulates your adrenal glands to produce cortisol (the “stress hormone”), which helps maintain blood pressure and blood glucose (blood sugar) levels.
• Follicle-stimulating hormone (FSH): This hormone stimulates sperm production in men and people assigned male at birth and stimulates the ovaries to produce estrogen and egg development in women and people assigned female at birth.
• Growth hormone (GH): In children, growth hormone stimulates growth. In adults, growth hormone helps maintain healthy muscles and bones and impacts fat distribution. GH also impacts your metabolism.
• Luteinizing hormone (LH): This hormone stimulates ovulation in people assigned female at birth and testosterone production in people assigned male at birth.
• Prolactin: This hormone stimulates breast milk production after giving birth and can affect menstrual periods in people assigned female at birth. It can affect fertility and sexual functions in adults.
• Thyroid-stimulating hormone (TSH): This hormone stimulates your thyroid to produce hormones that manage metabolism, energy and your nervous system.

Your pituitary gland stores and releases the following hormones, but your hypothalamus produces them:

• Antidiuretic hormone (ADH or vasopressin): This hormone regulates the water balance and sodium (salt) levels in your body.
• Oxytocin: In people assigned female at birth, oxytocin helps labor to progress during childbirth and causes breast milk (chest milk) to flow. It also influences the bonding between parent and baby.

Who does panhypopituitarism affect?

Panhypopituitarism can affect anyone at any age.

How common is panhypopituitarism?

Panhypopituitarism is rare. There are approximately four cases of panhypopituitarism per 100,000 people across the globe per year.

Is panhypopituitarism life-threatening?

Yes, panhypopituitarism can be life-threatening, especially if you have a significant deficiency of adrenocorticotropic hormone (ACTH or corticotropin).

Adrenal crisis (acute cortisol insufficiency) is a life-threatening complication of panhypopituitarism. The cause is a lack of ACTH, which is a hormone that controls your cortisol levels. It’s treatable but requires immediate medical treatment.

Signs and symptoms of adrenal crisis include:

• Fever.
• Weakness.
• Confusion.
• Low blood pressure (hypotension).
• Fast heart rate (tachycardia).
• Vomiting.
• Diarrhea.
• Low blood sugar (hypoglycemia).

If you or your child are experiencing these symptoms, call 911 or get to the nearest hospital as soon as possible.

What are the signs and symptoms of panhypopituitarism?

The signs and symptoms of panhypopituitarism vary widely based on how much of each of the pituitary hormones is lacking and whether the condition develops rapidly or slowly.

Symptoms of panhypopituitarism that children and adults can have include:

• Nausea or dizziness.
• Fatigue.
• Depression and/or anxiety.
• Frequent infections.
• Low blood sugar (hypoglycemia).
• Sensitivity to cold.
• Unusually dry skin.
• Unexplained weight loss or weight gain.
• Irregular lipid and cholesterol levels (dyslipidemia).
• Fast heart rate (tachycardia).
• Excessive thirst and excessive urination.
• Irregular menstruation (periods).
• Female infertility.
• Male infertility.

Additional symptoms of panhypopituitarism that are specific to infants, children and/or adolescents include:

• Prolonged jaundice in newborns.
• Small penis in infants assigned male at birth (micropenis).
• Slowed growth.
• Delayed puberty.

These symptoms may resemble other conditions or medical issues. It’s important to always consult your healthcare provider if you’re experiencing new or prolonged symptoms to get a proper diagnosis.

What causes panhypopituitarism?

Many conditions and situations can cause panhypopituitarism. In some cases, healthcare providers can’t determine the cause. This is called idiopathic panhypopituitarism.

In general, the cause of panhypopituitarism is some type of damage to your hypothalamus and/or pituitary gland that causes either or both of them to not function properly.

Understanding the possible causes of panhypopituitarism involves understanding how your hypothalamus and pituitary gland work together.

The hypothalamus-pituitary gland connection

Together, your pituitary gland and hypothalamus form a hypothalamus-pituitary complex that serves as your brain’s central command center to control vital bodily functions.

Your hypothalamus is the part of your brain that’s in charge of some of your body’s basic operations. It sends messages to your autonomic nervous system. Your hypothalamus also tells your pituitary gland to produce and release hormones that affect other areas of your body.

Your pituitary gland connects to your hypothalamus through a stalk of blood vessels and nerves (the pituitary stalk). Through that stalk, your hypothalamus communicates with your pituitary gland.

Your hypothalamus makes the following hormones to communicate with and stimulate your pituitary gland:

• Corticotropin-releasing hormone.
• Dopamine.
• Gonadotropin-releasing hormone.
• Growth hormone-releasing hormone.
• Somatostatin.
• Thyrotropin-releasing hormone.

Since your pituitary gland and hypothalamus work together so closely, if one of them becomes damaged, it can affect the hormonal function of the other. This can result in panhypopituitarism.

Pituitary-related causes of panhypopituitarism

Conditions or situations that can damage your pituitary gland and cause panhypopituitarism include:

• Pituitary adenomas (noncancerous tumors).
• Pituitary gland surgery, typically for adenoma removal.
• Radiation therapy for a pituitary adenoma.
• Pituitary apoplexy (sudden destruction of pituitary gland tissue due to a lack of blood supply to your pituitary or bleeding into your pituitary).
• Underdeveloped or poorly formed pituitary gland at birth.

Hypothalamus-related causes of panhypopituitarism

Conditions or situations that can damage your hypothalamus and cause panhypopituitarism include:

• Traumatic brain injury (TBI).
• Brain surgery on or near your hypothalamus.
• Benign (noncancerous) tumors that form in your hypothalamus, such as craniopharyngiomas.
• Malignant (cancerous) tumors that metastasize (spread) to your hypothalamus from cancer elsewhere in your body, such as lung and breast cancers.
• Pressure from hydrocephalus.
• Stroke.
• Tuberculous meningitis.

How is panhypopituitarism diagnosed?

If you’re experiencing symptoms of panhypopituitarism, your healthcare provider will ask detailed questions about your symptoms and medical history and perform a physical exam.

They’ll then order tests to confirm a panhypopituitarism diagnosis and/or to rule out other conditions that could be causing your symptoms.

What tests will be done to diagnose panhypopituitarism?

Healthcare providers typically order multiple tests to diagnose panhypopituitarism, including imaging and hormone levels tests.

Imaging tests for diagnosing panhypopituitarism

Since panhypopituitarism results from damage to your hypothalamus or pituitary gland, your provider may order the following imaging tests to help determine the cause of panhypopituitarism:

• Brain MRI (magnetic resonance imaging) scan: An MRI scan uses radio waves and strong magnets to create detailed images of the inside of your body. Brain MRIs are considered the best way to find pituitary tumors that could be causing panhypopituitarism. An MRI scan can also help identify other issues with your hypothalamus or pituitary gland.
• Brain CT (computed tomography) scan: A brain CT scan uses X-rays and a computer to create detailed images of your brain. Your provider may use this test to see if you have a brain tumor or pituitary adenoma (noncancerous tumor) that’s causing panhypopituitarism. A CT scan can also help identify other issues with your hypothalamus or pituitary gland.

Hormone tests for diagnosing panhypopituitarism

If you have symptoms of panhypopituitarism, your provider will need to measure the levels of all the hormones your pituitary gland releases to check how deficient each one is and to help rule out other conditions.

While some pituitary hormones normally maintain a fairly stable level in your bloodstream, other pituitary hormone levels normally vary widely throughout the day. Because of this, some hormone tests are simple blood tests and others are specialized stimulation tests.

Hormone level tests include:

• Blood tests: Pituitary gland hormones that providers can measure through simple blood tests include thyroid-stimulating hormone (TSH), prolactin, follicle-stimulating hormone (FSH) and luteinizing hormone (LH). They may also measure other hormones that your pituitary hormones affect, such as thyroxine, estrogen and testosterone.
• ACTH stimulation test: This test measures how well your adrenal glands respond to adrenocorticotropic hormone (ACTH). It involves an injection of synthetic ACTH and timed blood draws.
• Growth hormone (GH) stimulation test: For this test, your provider injects medicine that would normally stimulate your pituitary gland to release growth hormone. They then take samples of your blood to measure your GH levels.
• Insulin tolerance test: An insulin tolerance test can help diagnose GH and ACTH deficiencies.

How is panhypopituitarism treated?

The treatment for panhypopituitarism greatly depends on how deficient each pituitary hormone is and the cause of the condition. Because of this, treatment is very individualized. Your healthcare team will determine what the best treatment plan is for you. Common treatment options for panhypopituitarism include:

• Hormone replacement therapy: Hormone replacement therapy aims to restore the deficient pituitary hormones to healthy levels. People with panhypopituitarism require lifetime hormone replacement therapy unless the underlying cause of the condition is reversible or treatable. Some hormone replacement medications are pills and others are injections (shots).
• Surgery: If you have a brain tumor or pituitary adenoma that’s causing panhypopituitarism, your provider may recommend surgery to remove the tumor or adenoma.
• Radiation therapy: If you have a brain tumor or pituitary adenoma that’s causing panhypopituitarism, your provider may recommend radiation therapy to treat and/or shrink the tumor.
• Corticosteroids: You may need increased doses of corticosteroids before and/or during any stressful event, whether physical or emotional. Physical stressors include illness, infection and surgery. Corticosteroids replace the adrenal hormones that aren't being produced because of an adrenocorticotropic hormone (ACTH) deficiency. Your body needs these hormones when it’s under stress.

Is panhypopituitarism curable?

In some cases, panhypopituitarism is reversible by treatment of the underlying cause, such as surgically removing a pituitary adenoma that was compressing the pituitary gland without causing damage. But in most cases, the hormone deficiencies from panhypopituitarism require lifelong treatment.

How can I prevent panhypopituitarism?

In most cases, you can’t prevent panhypopituitarism. But there are ways to catch it in its early phase if you’re at risk for developing it.

If you’ve experienced any of the following situations, you’re at greater risk for developing panhypopituitarism:

• Brain or pituitary gland surgery.
• Radiation therapy for your brain and/or pituitary gland.
• Traumatic brain injury.
• Hydrocephalus (buildup of fluid in your brain).

Your healthcare provider will likely recommend regular testing to check the function and health of your pituitary gland and/or hypothalamus if you’re at a greater risk for developing panhypopituitarism.

What is the prognosis (outlook) for panhypopituitarism?

The prognosis (outlook) for panhypopituitarism depends on several factors, including:

• How much of each of your pituitary hormones is lacking (deficient).
• Your age at the onset (beginning) of panhypopituitarism.
• If panhypopituitarism develops slowly or rapidly.
• How quickly you get a diagnosis and receive treatment.

Panhypopituitarism is associated with significant decreases in quality of life and life expectancy.

People with panhypopituitarism often develop obesity, decreased lean body mass and an increased risk of cardiovascular disease. People with panhypopituitarism may also have an increased risk of osteoporosis and bone fractures.

Careful, thorough treatment with hormone replacements and aggressive monitoring and treatment for cardiovascular disease risk factors may improve outcomes.

When should I see my healthcare provider for panhypopituitarism?

If you have symptoms of panhypopituitarism or received a diagnosis of the condition, you’ll likely need to see an endocrinologist — a healthcare provider who specializes in treating hormone-related conditions.

You’ll need to see your endocrinologist regularly throughout your life to ensure that your hormone replacement therapy is working well and to prevent excessive hormone replacement.

A note from Cleveland Clinic

A new diagnosis can be scary, but don’t be afraid to ask your healthcare provider questions about panhypopituitarism. Most cases of panhypopituitarism require lifelong treatment and monitoring of your hormones, so it’s important to see your provider regularly. Be sure to contact your provider if you have new or concerning symptoms. They’re available to help.