Diagnosis and Treatment of Pituitary Adenomas

Posted on February 8, 2017 by MaryO
A Review
Mark E. Molitch, MD1
Author Affiliations
JAMA. 2017;317(5):516-524. doi:10.1001/jama.2016.19699

Importance  Pituitary adenomas may hypersecrete hormones or cause mass effects. Therefore, early diagnosis and treatment are important.

Observations  Prevalence of pituitary adenomas ranges from 1 in 865 adults to 1 in 2688 adults. Approximately 50% are microadenomas (<10 mm); the remainder are macroadenomas (≥10 mm).

Mass effects cause headache, hypopituitarism, and visual field defects. Treatments include transsphenoidal surgery, medical therapies, and radiotherapy. Prolactinomas account for 32% to 66% of adenomas and present with amenorrhea, loss of libido, galactorrhea, and infertility in women and loss of libido, erectile dysfunction, and infertility in men; they are generally treated with the dopamine agonists cabergoline and bromocriptine.

Growth hormone–secreting tumors account for 8% to 16% of tumors and usually present with enlargement of the lips, tongue, nose, hands, and feet and are diagnosed by elevated insulin-like growth factor 1 levels and growth hormone levels; initial treatment is surgical. Medical therapy with somatostatin analogues, cabergoline, and pegvisomant is often also needed.

Adrenocorticotropic hormone (ACTH)–secreting tumors account for 2% to 6% of adenomas and are associated with obesity, hypertension, diabetes, and other morbidity. Measurement of a late-night salivary cortisol level is the best screening test but petrosal sinus sampling for ACTH may be necessary to distinguish a pituitary from an ectopic source.

The primary treatment of Cushing disease (hypercortisolism due to ACTH-producing adenomas, which is the cause in approximately 65% of the cases of hypercortisolism) is adenoma resection and medical therapies including ketoconazole, mifepristone, and pasireotide.

Hyperthyroidism due to thyroid-stimulating hormone–secreting tumors accounts for 1% of tumors and is treated with surgery and somatostatin analogues if not surgically cured. Clinically nonfunctioning adenomas account for 15% to 54% of adenomas and present with mass effects; surgery is generally required, although incidentally found tumors can be followed if they are asymptomatic.

Conclusions and Relevance  Patients with pituitary adenomas should be identified at an early stage so that effective treatment can be implemented. For prolactinomas, initial therapy is generally dopamine agonists. For all other pituitary adenomas, initial therapy is generally transsphenoidal surgery with medical therapy being reserved for those not cured by surgery.

Read the full text here: http://jamanetwork.com/journals/jama/article-abstract/2600472

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Exophthalmos and Cushing’s Syndrome

Posted on February 4, 2015 by MaryO

A woman experienced red, irritated and bulging eyes. She saw an ophthalmologist who strongly suspected Graves’ ophthalmopathy. However, the patient did not have and never had hyperthyroidism.

Indeed, she had primary hypothyroidism optimally treated with levothyroxine. Her thyroid stimulating hormone level was 1.197 uIU/mL.

An MRI of the orbits showed normal extraocular muscles without thickening, but there was mild proptosis and somewhat increased intraorbital fat content. Both thyroid-stimulating immunoglobulins as well as thyrotropin receptor antibodies were negative.

The patient presented to her primary care physician a few months later. She had experienced a 40-lb weight gain over only a few months and also had difficult-to-control blood pressure.

After failing to respond to several antihypertensive medications, her primary care physician astutely decided to evaluate for secondary causes of hypertension. A renal ultrasound was ordered to evaluate for renal artery stenosis, and the imaging identified an incidental right-sided adrenal mass. A CT confirmed a 3.4-cm right-sided adrenal mass. Her morning cortisol was slightly high at 24.7 ug/dL (4.3 – 22.4) and her adrenocorticotropic hormone was slightly low at 5 pg/mL (10-60).

At this point I saw the patient in consultation. She definitely had many of the expected clinical exam findings of Cushing’s syndrome, including increased fat deposition to her abdomen, neck, and supraclavicular areas, as well as striae. Her 24-hour urine cortisol was markedly elevated at 358 mcg/24hrs (< 45) confirming our suspicions.

She asked me, “Do you think that my eye problem could be related to this?”

“I’ve not heard of it before,” I replied, “but that doesn’t mean there can’t be a connection. Wouldn’t it be wonderful if your eyes got better after surgery?”

The patient underwent surgery to remove what fortunately turned out to be a benign adrenal adenoma.

When we saw her in follow-up 2 weeks later, her blood pressures were normal off medication and her eye symptoms had improved. I had a medical student rotating with me, so I suggested that we do a PubMed literature search.

The first article to come up was a case report titled “Exophthalmos: A Forgotten Clinical Sign of Cushing’s Syndrome.” Indeed, not only did Harvey Cushing describe this clinical finding in his original case series in 1932, but others have reported that up to 45% of patients with active Cushing’s syndrome have exophthalmos.

The cause is uncertain but is theorized to be due to increased intraorbital fat deposition. Unlike exophthalmos due to thyroid disease, the orbital muscles are relatively normal — just as they were with our patient.

Some of you may have seen exophthalmos in your Cushing’s patients; however, this was the first time I had seen it. Just because one has not heard of something, does not mean it could never happen; no one knows everything. “When in doubt, look it up” is a good habit for both attending physicians and their students.

For more information:

Giugni AS, et al. Case Rep Endocrinol. 2013; 2013: 205208.

From http://www.healio.com/endocrinology/adrenal/news/blogs/%7B779bf3e5-e1da-459e-af27-955c9b4274a5%7D/thomas-b-repas-do-facp-face-cde/exophthalmos-and-cushings-syndrome

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Classifying hypertension

Posted on May 19, 2013 by MaryO

HYPERTENSION is classified into two categories according to its cause: essential and secondary.

The vast majority of patients have essential or primary hypertension, while only about 5-10% of patients have secondary hypertension, which are mainly caused by kidney and hormonal conditions like renal artery stenosis, hyperthyroidism, Cushing’s syndrome, and even pregnancy, among others.

The exact cause of essential hypertension is still unknown, although it is certainly the result of a combination of factors, including increasing age, having relatives with high blood pressure (ie family history), a sedentary lifestyle, a poor diet with too much salt, drinking too much alcohol, smoking and too much stress.

Says Malaysian Society of Hypertension president and Universiti Malaya Department of Primary Care Medicine senior consultant Prof Datin Dr Chia Yook Chin: “Each factor increases blood pressure by just a little, but when you add them all together little by little, it raises it by quite a lot.”

Despite not knowing the root cause of hypertension, it has been established that there is overstimulation of the sympathetic nerves in people with this condition.

This in turn increases the secretion of certain hormones involved in the regulation of sodium and fluids in the body, called renin, angiotensin, and aldosterone.

The amount of salt and water in our body affects our blood pressure – the more salt and water present, the higher our blood pressure.

These two elements are regulated by our kidneys through the three hormones mentioned above, which are produced by the adrenal glands located on top of the kidneys.

The overstimulation of the sympathetic nerves also results in increased vascular tone, which causes our arteries to become constricted, thus, also raising blood pressure.

From The Star

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Hyperthyroidism due to thyroid stimulating hormone secretion after surgery for Cushing’s syndrome: A novel cause of the syndrome of inappropriate secretion of thyroid stimulating hormone

Posted on May 14, 2013 by MaryO
Overview of the thyroid system (See Wikipedia:...

Overview of the thyroid system (See Wikipedia:Thyroid). To discuss image, please see Talk:Human body diagrams (Photo credit: Wikipedia)

  1. Daisuke Tamada, MD1,
  2. Toshiharu Onodera, MD1,
  3. Tetsuhiro Kitamura, MD, PhD1,
  4. Yuichi Yamamoto, MD1,
  5. Yoshitaka Hayashi, MD, PhD2,
  6. Yoshiharu Murata, MD, PhD2,
  7. Michio Otsuki, MD, PhD1 and
  8. Iichiro Shimomura, MD, PhD1

Author Affiliations


  1. 1Department of Metabolic Medicine, Osaka University Graduate School of Medicine, Osaka, Japan

  2. 2Department of Genetics, Research Institute of Environmental Medicine, Nagoya University, Nagoya, Japan
  1. Address all correspondence and requests for reprints to: Michio Otsuki, MD, PhD, Department of Metabolic Medicine, Osaka University Graduate School of Medicine, 2–2 Yamada-oka, Suita, Osaka 565-0871, Japan, Telephone: +81-6-6879-3732. Fax: +81-6-6879-3739, E-mail: otsuki@endmet.med.osaka-u.ac.jp.

Abstract

Context: Hyperthyroidism with the syndrome of inappropriate secretion of thyroid stimulating hormone (TSH) (SITSH) occurred by a decrease in hydrocortisone dose after surgery for Cushing’s syndrome. This is a novel cause of SITSH.

Objective: The aim of this study was to describe and discuss the two cases of SITSH patients who were found after surgery for Cushing’s syndrome. We also checked whether SITSH occurred in the consecutive 7 patients with Cushing’s syndrome after surgery.

Patients and Methods: A 45-year-old Japanese woman with adrenocorticotropin (ACTH)-independent Cushing’s syndrome and a 37-year-old Japanese man with ACTH-dependent Cushing’s syndrome presented SITSH caused by insufficient replacement of hydrocortisone for postoperative adrenal insufficiency. When the dose of hydrocortisone was reduced to less than 20 mg/day within 18 days after operation, SITSH occurred in both cases. We examined whether the change of the hydrocortisone dose induced the secretion of TSH. Free T3 and TSH were normalized by the hydrocortisone dose increase of 30 mg/day and these were elevated by the dose decrease of 10 mg/day. We also checked TSH and thyroid hormone the consecutive 7 patients with Cushing’s syndrome after surgery. Six (66.6 %) of nine patients showed SITSH.

Conclusions: This is the first report that insufficient replacement of hydrocortisone after the surgery of Cushing’s syndrome caused SITSH. Hyperthyroidism by SITSH as well as adrenal insufficiency can contribute to withdrawal symptoms of hydrocortisone replacement. So we need to consider the possibility of SITSH for the pathological evaluation of withdrawal syndrome of hydrocortisone replacement.

  • Received May 4, 2013.
  • Accepted May 8, 2013.

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