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Endocrine Abstracts (2024) 100 WD1.3 | DOI: 10.1530/endoabs.100.WD1.3

SFEEU2024 Society for Endocrinology Clinical Update 2024 Workshop D: Disorders of the adrenal gland (20 abstracts)

A case of ACTH independent Cushing’s syndrome with bilateral adrenal nodules

Gillian Bennett & Raj Tanday


King George Hospital, London, United Kingdom


Case presentation: A 40 year old woman with a history of epilepsy was referred to the endocrine clinic with hypertension and significant weight gain (BMI 42.3 kg/m2). She had a sleeve gastrectomy performed in Turkey several years previously, resulting in an 18 kg weight loss. However, she regained this weight, most of which occurred in the six months prior to presentation. She had also noted the recent development of striae and was complaining of myopathy, so severe that she struggled to get out of bed each morning. As she clinically appeared Cushingoid and as a result a number of screening tests were carried out, including a low dose dexamethasone suppression test (cortisol was 143 nmol/l) 24 hour urine free cortisol (1406 nmol/24 hours), midnight salivary cortisone (95.8 nmol/l). Given the fact that she failed her screening tests, an ACTH was sent and found to be suppressed (ACTH <3 ng/l), this all supported a diagnosis of ACTH independent Cushing’s Syndrome. A CT of her adrenals was carried and discussed at the surgical/endocrine MDT, it was determined that there were bilateral adrenal nodules these measured 2.1 cm on the right and 3 cm on the left. Metyrapone has been added to help with the symptoms of hypercortisolaemia and the MDT are in discussions regarding the best course of treatment for this lady. Currently the plan is to carry out sequential adrenalectomies starting with the larger adenoma (left). Postoperatively her response will be monitored but may possibly require a right sided adrenalectomy.

Fig.1: Cortisol day curve prior to commencement of metyrapone
TimeCortisol
09:00717
10:00684
11:00656
12:00673
13:00749
14:00681

Discussion: This case has proven challenging as her clinicians are uncertain if one or both of her adrenals are contributing to her Cushing’s syndrome, if this could be determined and she is found to have one normal adrenal gland she may avoid lifelong steroid replacement and the morbidity attached to this. On review of the literature there have been cases where adrenal vein sampling (AVS) has been used to localise hypercortisolaemia, however these are primarily case reports[1–4]. Raj et al has developed an algorithm to help localise the cortisol-producing adenoma in subclinical Cushing’s disease using an adrenal vein to peripheral vein ratio comparing cortisol and metanephrine gradients[4]. Perhaps future research could be carried out investigating the feasibility of AVS in the diagnosis and lateralisation of cortisol-producing adrenal adenomas.

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