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

A Subtle Case of Cushing’s

English: "Dr. Harvey Cushing," oil o...

English: “Dr. Harvey Cushing,” oil on canvas, by the American artist Edmund Tarbell. Courtesy of the Dittrick Medical History Center. (Photo credit: Wikipedia)

Synopsis: 36 year old male who presented with weight gain, proximal muscle weakness, and excessive sweating. Among multiple 24-hour urine free cortisol and midnight salivary tests, a minority were minimally elevated.

The diagnosis of Cushing’s disease was questioned over an eight month evaluation period.

The challenges of diagnosing Cushing’s disease are discussed.

Clinical History

Pre-Operative Imaging

Operative Findings

Surgical Pathology

Post-Operative Course

Discussion, Part 1

Discussion, Part 2


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From UCLA Pituitary Tumor Program

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