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

Topical Steroid Use in Psoriasis Patient Leads to Severe Adrenal Insufficiency

This article is written live from the American Association of Clinical Endocrinologists (AACE) 2017 Annual Meeting in Austin, TX. MPR will be reporting news on the latest findings from leading experts in endocrinology. Check back for more news from AACE 2017.

 

At the AACE 2017 Annual Meeting, lead study author Kaitlyn Steffensmeier, MS III, of the Dayton Veterans Affairs (VA) Medical Center, Dayton, OH, presented a case study describing a patient “who developed secondary adrenal insufficiency secondary to long-term topical steroid use and who with decreased topical steroid use recovered.”

The patient was a 63-year-old white male with a 23-year history of psoriasis. For 18 years, the patient had been applying Clobetasol Propionate 0.05% topically on several areas of his body every day. Upon presentation to the endocrine clinic for evaluation of his low serum cortisol, the patient complained of a 24-pound weight gain over a 2-year period, feeling fatigued, as well as facial puffiness.

Laboratory analysis found that the patient’s random serum cortisol and ACTH levels were low (0.2µg/dL and <1.1pg/mL, respectively). According to the study authors, “the labs were indicative of secondary adrenal insufficiency.” Additionally, a pituitary MRI “showed a 2mm hypoenhancing lesion within the midline of the pituitary gland consistent with Rathke’s cleft cyst versus pituitary microadenoma.”

The patient was initiated on 10mg of hydrocortisone in the morning and 5mg in the evening and was instructed to decrease the use of his topical steroid to one time per month. For the treatment of his psoriasis, the patient was started on apremilast, a phosphodiesterase-4 enzyme (PDE4) inhibitor, and phototherapy.

After 2.5 years, the patient had a subnormal response to the cosyntropin stimulation test. However, after 3 years, a normal response with an increase in serum cortisol to 18.7µg/dL at 60 minutes was obtained; the patient was then discontinued on hydrocortisone. Additionally, a stable pituitary tumor was shown via a repeat pituitary MRI.

The study authors explained that, although secondary adrenal insufficiency is not commonly reported, “one study showed 40% of patients with abnormal cortisol response to exogenous ACTH after two weeks of topical glucocorticoids usage.” Another meta-analysis of 15 studies (n=320) revealed 4.7% of patients developing adrenal insufficiency after using topical steroids. Because of this, “clinicians need to be aware of potential side effects of prolong topical steroid use,” added the study authors.

For continuous endocrine news coverage from the AACE 2017 Annual Meeting, check back to MPR’s AACE page for the latest updates.

From http://www.empr.com/aace-2017/topical-steroid-psoriasis-clobestasol-propionate/article/654335/

Comparison of MRI techniques for detecting microadenomas in Cushing’s disease

1Department of Neurological Surgery and 2Department of Radiology, University of Virginia Health Science Center, University of Virginia, Charlottesville, Virginia
ABBREVIATIONS ACTH = adrenocorticotropic hormone; CMRI = conventional MRI; DMRI = dynamic contrast-enhanced MRI; FSH = follicle-stimulating hormone; IPSS = inferior petrosal sinus sampling; SE = spin echo; SGE = spoiled-gradient echo 3D T1 sequence; SPGR = spoiled gradient–recalled acquisition; VIBE = volumetric interpolated breath-hold examination.

INCLUDE WHEN CITING Published online April 28, 2017; DOI: 10.3171/2017.3.JNS163122.

Correspondence Edward H. Oldfield, Department of Neurological Surgery, University of Virginia, Box 800212, Charlottesville, VA 22908. email: .
OBJECTIVE

Many centers use conventional and dynamic contrast-enhanced MRI (DMRI) sequences in patients with Cushing’s disease. The authors assessed the utility of the 3D volumetric interpolated breath-hold examination, a spoiled-gradient echo 3D T1 sequence (SGE) characterized by superior soft tissue contrast and improved resolution, compared with DMRI and conventional MRI (CMRI) for detecting microadenomas in patients with Cushing’s disease.

METHODS

This study was a blinded assessment of pituitary MRI in patients with proven Cushing’s disease. Fifty-seven patients who had undergone surgery for Cushing’s disease (10 male, 47 female; age range 13–69 years), whose surgical findings were considered to represent a microadenoma, and who had been examined with all 3 imaging techniques were included. Thus, selection emphasized patients with prior negative or equivocal MRI on referral. The MRI annotations were anonymized and 4 separate imaging sets were independently read by 3 blinded, experienced clinicians: a neuroradiologist and 2 pituitary surgeons.

RESULTS

Forty-eight surgical specimens contained an adenoma (46 ACTH-staining adenomas, 1 prolactinoma, and 1 nonfunctioning microadenoma). DMRI detected 5 adenomas that were not evident on CMRI, SGE detected 8 adenomas not evident on CMRI, including 3 that were not evident on DMRI. One adenoma was detected on DMRI that was not detected on SGE. McNemar’s test for efficacy between the different MRI sets for tumor detection showed that the addition of SGE to CMRI increased the number of tumors detected from 18 to 26 (p = 0.02) based on agreement of at least 2 of 3 readers.

CONCLUSIONS

SGE shows higher sensitivity than DMRI for detecting and localizing pituitary microadenomas, although rarely an adenoma is detected exclusively by DMRI. SGE should be part of the standard MRI protocol for patients with Cushing’s disease.

Full text at http://thejns.org/doi/full/10.3171/2017.3.JNS163122

Adrenocortical carcinoma masquerading as Cushing’s disease

BMJ Case Reports 2017; doi:10.1136/bcr-2016-217519

Summary

Cushing’s syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels.

However, 30% of the patients with CS have ACTH levels in the ‘grey zone’ (5–20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing’s syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing’s disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3–27% of the healthy population. Therefore, in a patient with CS with equivocal ACTH levels and a pituitary microadenoma, multiple samplings for ACTH and adrenal imaging should be performed to exclude ACTH-independent CS and if required, bilateral inferior petrosal sinus sampling to determine the source of ACTH excess.

Find the entire article here: http://casereports.bmj.com/content/2017/bcr-2016-217519.full

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

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