An unusual case of Cushing’s syndrome due to bihormonal ACTH–prolactin secreting pituitary macroadenoma with rapid response to cabergoline

  1. Shalini Kunasegaran1,2,
  2. Michael S Croxson1,
  3. Ian Holdaway1,
  4. Rinki Murphy1

+Author Affiliations


  1. 1Department of EndocrinologyAuckland District Health BoardAuckland, New Zealand

  2. 2Department of EndocrinologyWaitemata District Health BoardTakapuna, New Zealand
  1. Correspondence to Dr Shalini Kunasegaran, shal84@gmail.com
  • Accepted 13 July 2017
  • Published 7 August 2017

Summary

A 23-year-old man presenting with florid Cushing’s syndrome was found to have high plasma ACTH and very high serum prolactin. Pituitary MRI showed a large invasive macroadenoma.

Low-dose cabergoline promptly suppressed both ACTH and prolactin levels within 2 weeks, with unexpected clinical and biochemical hypocortisolism requiring hydrocortisone replacement. Secondary hypogonadism was reversed. Clinical and biochemical remission of his Cushing’s syndrome together with significant shrinkage of his macroadenoma has been maintained for 1 year on cabergoline 0.5 mg twice weekly. Reduction in pituitary

Reduction in pituitary tumour volume and brisk fall in serum prolactin in response to low-dose cabergoline is regularly observed in patients with macroprolactinomas, but the concurrent fall in the plasma ACTH level and hypocortisolism was a pleasant surprise.

We assume that he most likely has a single bihormonal adenoma that is enriched with dopamine-2 receptors.

From http://casereports.bmj.com/content/2017/bcr-2017-219921.short?rss=1

Pituitary Issues: Irregular Periods

Q: I am 28 years old and I have not yet started my periods naturally. I have to take medicine for periods — Novelon. The doctors say that there is some problem with my hormones in the pituitary gland. Please advise me how to get normal and natural periods, because after taking the medicine I get my period, but without medicines I don’t.

A by Dr Sharmaine Mitchell: The problem you have with your menstrual period being irregular is most likely due to overproduction of the hormone prolactin by the pituitary gland in the brain. The pituitary gland can sometimes enlarge and cause an overproduction of prolactin and this can result in inappropriate milk production in the breasts (white nipple discharge), irregular menstruation or absent menstrual periods, headaches and blurred vision. The blurred vision occurs as a result of compression of the optic nerve which supplies the eyes, by the enlarged brain tumour in the pituitary gland.

You should get a magnetic resonance imaging (MRI) or CT scan of the brain and pituitary gland done. You should also test your prolactin levels to determine the extent of overproduction of the hormone.

Other investigations should include a thyroid function test (TSH), follicle stimulating hormone (FSH) and leutinizing hormone (LH), and baseline testosterone level tests.

Abnormalities in the production of thyroid hormones can also cause menstrual irregularities and this should be ruled out.

Polycystic ovarian disease can also cause irregular menstrual periods and checking the level of FSH, LH and testosterone will help to rule out this diagnosis. This condition is usually associated with excessive weight gain, abnormal male pattern distribution on the face, chest and abdomen and an increased risk for diabetes mellitus. A pelvic ultrasound to look at the structure of the ovaries and to rule out polycystic ovaries is essential.

If the pituitary gland is enlarged, then medication can be prescribed to shrink it. Bromocriptine or Norprolac are commonly used drugs which work well in reducing the prolactin levels and establishing regular menstrual cycles. The use of these drugs will also help to establish ovulation and improve your fertility.

In some cases it may become necessary to have surgery done if the tumour in the pituitary gland is large and does not respond to the usual medications prescribed to shrink the pituitary gland. The MRI of the brain and pituitary gland will give an idea as to the size of the gland and help to determine if there is a need for you to see the neurosurgeon.

In most cases medical management with drugs will work well and there is no need for surgical intervention. This is a problem that can recur, so it may be necessary to take treatment intermittently for a long period of time, especially if fertility is desired.

Consult your doctor who will advise you further. Best wishes.

Dr Sharmaine Mitchell is an obstetrician and gynaecologist. Send questions via e-mail to allwoman@jamaicaobserver.com; write to All Woman, 40-42 1/2 Beechwood Ave, Kingston 5; or fax to 968-2025. All responses are published. Dr Mitchell cannot provide personal responses.

DISCLAIMER:

The contents of this article are for informational purposes only and must not be relied upon as an alternative to medical advice or treatment from your own doctor.

From http://www.jamaicaobserver.com/magazines/allwoman/Still-no-normal-period-at-28_87596

The Pituitary Gland: Small But Mighty

The pituitary gland works hard to keep you healthy, doing everything from ensuring proper bone and muscle growth to helping nursing mothers produce milk for their babies. Its functionality is even more remarkable when you consider the gland is the size of a pea.

“The pituitary is commonly referred to as the ‘master’ gland because it does so many important jobs in the body,” says Karen Frankwich, MD, a board-certified endocrinologist at Mission Hospital. “Not only does the pituitary make its own hormones, but it also triggers hormone production in other glands. The pituitary is aided in its job by the hypothalamus. This part of the brain is situated above the pituitary, and sends messages to the gland on when to release or stimulate production of necessary hormones.”

These hormones include:

  • Growth hormone, for healthy bone and muscle mass
  • Thyroid-stimulating hormone, which signals the thyroid to produce its hormones that govern metabolism and the body’s nervous system, among others
  • Follicle-stimulating and luteinizing hormones for healthy reproductive systems (including ovarian egg development in women and sperm formation in men, as well as estrogen and testosterone production)
  • Prolactin, for breast milk production in nursing mothers
  • Adrenocorticotropin (ACTH), which prompts the adrenal glands to produce the stress hormone cortisol. The proper amount of cortisol helps the body adapt to stressful situations by affecting the immune and nervous systems, blood sugar levels, blood pressure and metabolism.
  • Antidiuretic (ADH), which helps the kidneys control urine levels
  • Oxytocin, which can stimulate labor in pregnant women

The work of the pituitary gland can be affected by non-cancerous tumors called adenomas. “These tumors can affect hormone production, so you have too little or too much of a certain hormone,” Dr. Frankwich says. “Larger tumors that are more than 1 centimeter, called macroadenomas, can also put pressure on the area surrounding the gland, which can lead to vision problems and headaches. Because symptoms can vary depending on the hormone that is affected by a tumor, or sometimes there are no symptoms, adenomas can be difficult to pinpoint. General symptoms can include nausea, weight loss or gain, sluggishness or weakness, and changes in menstruation for women and sex drive for men.”

If there’s a suspected tumor, a doctor will usually run tests on a patient’s blood and urine, and possibly order a brain-imaging scan. An endocrinologist can help guide a patient on the best course of treatment, which could consist of surgery, medication, radiation therapy or careful monitoring of the tumor if it hasn’t caused major disruption.

“The pituitary gland is integral to a healthy, well-functioning body in so many ways,” Dr. Frankwich says. “It may not be a major organ you think about much, but it’s important to know how it works, and how it touches on so many aspects of your health.”

Learn more about Mission Hospital. Learn more about Dr. Frankwich.

From http://www.stjhs.org/HealthCalling/2016/December/The-Pituitary-Gland-Small-but-Mighty.aspx

Hypopituitarism – Deficiency in Pituitary Hormone Production

By Yolanda Smith, BPharm

Hypopituitarism is a health condition in which there is a reduction in the production of hormones by the pituitary gland.

The pituitary gland is located at the base of the brain and is responsible for the production of several hormones, including:

  • Adrenocorticotropic hormone (ACTH), which controls the production of the vital stress hormones cortisol and dehydroepiandrosterone (DHEA) in the adrenal gland
  • Thyroid stimulating hormone (TSH), which controls the production of hormones by the thyroid gland
  • Luteinizing hormone (LH) and follicle-stimulating hormone (FSH), which control the secretion of the primary sex hormones and affect fertility
  • Growth hormone (GH), which regulates the growth processes in childhood and other metabolic processes throughout life
  • Prolactin (PRL), which facilitates the production of breast milk
  • Oxytocin, which is crucial during labor, childbirth and lactation
  • Antidiuretic hormone (ADH), also known as vasopressin, which regulates the retention of water and the blood pressure

An individual with hypopituitarism shows a deficiency in one or more of these hormones. This inevitably leads to abnormal body function, as an effect of the low levels of the hormone in the body, and may result in symptoms.

Causes

Hypopituitarism is most commonly due to the destruction, compression or inflammation of pituitary tissue by a brain tumor in that region. Other causes include:

  • Head injury
  • Infections such as tuberculosis
  • Ischemic or infarct injury
  • Radiation injury
  • Congenital and genetic causes
  • Infiltrative diseases such as sarcoidosis

Symptoms

General symptoms that are associated with pituitary hormone deficiency include:

  • Weakness and fatigue
  • Decreased appetite
  • Weight loss
  • Sensitivity to cold
  • Swollen facial features or body

There are also likely to be more specific symptoms according to the type of pituitary hormone deficiency, such as:

  • ACTH deficiency:
    • abdominal pain
    • low blood pressure
    • low serum sodium levels
    • skin pallor
  • TSH deficiency:
    • generalized body puffiness
    • sensitivity to cold
    • constipation
    • impaired memory and concentration
    • dry skin
    • anemia
  • LH and FSH deficiency:
    • reduction in libido
    • erectile dysfunction in men
    • abnormal menstrual periods
    • vaginal dryness in women
    • difficulty in conceiving
    • infertility.
  • GH deficiency:
    • slow growth
    • short height
    • an increase in body fat

Treatment

The first step in the treatment of hypopituitarism is to identify the cause of the condition.

Secondly, the hormones that are deficient must be identified. From this point, the appropriate treatment decisions can be made to promote optimal patient outcomes.

Hormone replacement therapy is the most common type of treatment for a patient with hypopituitarism.

This may involve supplementation of one or more hormones that are deficient, to reduce or correct the impact of the deficiency.

Follow Up

As hormone replacement therapy is expected to continue on a lifelong basis, it is important that patients have a good understanding of the therapy.

It is especially important to educate patients on what to do in case of particular circumstances that may change their hormone requirements.

For example, during periods of high stress, the demand for many hormones is increased, and the dose of hormone replacement may need to be adjusted accordingly.

It is recommended that patients have regular blood tests to monitor their hormone levels and ensure that they are in the normal range.

Patients should also carry medical identification, such as a medical bracelet or necklace, to show that they are affected by hypopituitarism and inform others about their hormone replacement needs and current treatment. This can help to meet their medical needs in case of any emergency.

Epidemiology

Hypopituitarism is a rare disorder that affects less than 200,000 individuals in the United States, with an incidence of 4.2 cases per 100,000 people per year.

The incidence is expected to be higher in certain subsets of the population, such as those that have suffered from a brain injury. Statistics in reference to these population groups have not yet been determined.

Reviewed by Dr Liji Thomas, MD.

References

From http://www.news-medical.net/health/Hypopituitarism-Deficiency-in-Pituitary-Hormone-Production.aspx

Pituitary Incidentaloma Treatment Guideline

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It is unclear how many people have pituitary incidentaloma, but imaging and autopsy studies indicate they are quite common and occur in up to one-third of patients. Fortunately, the vast majority of these serendipitously discovered tumors are clinically insignificant.

A management guideline in the Annals of Endocrinology brings endocrinologists up to date on current thinking about pituitary incidentaloma management.   Endocrinologists classify these tumors as micro- or macro-. Microincidentalomas are discovered in around 10% of patients, often upon CT after a fall, and are less than 1 cm in diameter. They may grow, but only 5% proceed to macroincidentaloma.

Currently, experts recommend assessing nonfunctioning (NF) microincidentaloma clinically for signs of hypersecretion (hyperprolactinemia, acromegaly or Cushing’s syndrome), with subsequent systematic prolactin and IGF-1 assay.   Pituitary incidentalomas that are larger than 1 cm at discovery—macroincidentalomas—are more likely to grow, with 25% and 24%-40% of patients having larger tumors at 4 and 8 years after diagnosis respectively.

Concerns escalate and closer surveillance is needed if a macroadenoma is in contact with the optic chiasm. With any NF macroincidentaloma, experts recommend assessing patients for signs of hormonal hypersecretion or hypopituitarism. Then, laboratory screening for hypersecretion or hormonal deficiency is needed, as is ophthalmologic assessment (visual acuity and visual field) if the lesion is near the optic chiasm (OC).   Surveillance differs by tumor size, with 5 mm the cutoff for NF microincidentaloma.

Tumors smaller than that require no surveillance, and those larger need to be monitored with MRI at 6 months and then 2 years. Endocrinologists should revisit macroincidentaloma distant from the optic chiasm with MRI at 1 year and conduct hormonal exploration (for anterior pituitary deficiency), then monitor every 2 years.   Proximity to the optic chiasm often creates a need for surgery or increased vigilance. MRI is recommended at 6 months, with hormonal and visual assessment, then annual MRI and hormonal and visual assessment every 6 months.

Specific types of pituitary incidentaloma call for surgery: evolutive NF microincidentaloma, NF macroincidentaloma associated with hypopituitarism or showing progression, incidentaloma compressing the optic chiasm, possible malignancy, non-compliant patient, pregnancy desired in the short-term, or context at risk of apoplexy.

Few guidelines are published for pituitary incidentaloma, and this one is enhanced with a decision tree that walks endocrinologist through the recommendations. –

See more at: http://www.hcplive.com/medical-news/pituitary-incidentaloma-treatment-guideline#sthash.0DqxeTru.dpuf

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