E-Poster Presentation ESA-SRB-ANZBMS 2021

A Tale of Two Adenomas: A Case of Autonomous Cortisol Secretion (#369)

Rebekah R Chew 1 , Jui T Ho 1
  1. Southern Adelaide Local Health Network, Bedford Park, SA, Australia

A 37 year-old lady was found to have bilateral adrenal incidentalomas. Clinically, she was obese (BMI 36), normotensive, and with no cushingoid features. CT Abdomen showed bilateral hypodense adrenal lesions, measuring 2.1 x 1.4 cm on the right and 2.6 x 1.7 cm on the left. Initial investigation showed no evidence of hormonal hypersecretion, except for a suppressed ACTH.

 

The patient was under regular clinical, biochemical and radiological surveillance for the next ten years. She developed significant weight gain, hypertension, and osteopenia of the spine with Z-score -1.9 at L3-L4 (pre-menopausal). Repeat testing showed abnormal cortisol post 1 mg dexamethasone suppression test of 101 nmol/L, suppressed ACTH and DHEAS but no overt Cushing’s syndrome. Midnight salivary cortisol and 24 hour urinary free cortisol measurements were normal. 

 

Both adrenal adenomas had increased by 30% and 50% from baseline (Figure 1). Norcholesterol scan confirmed hyperfunctioning of the left adrenal lesion for which she underwent left adrenalectomy. Histology showed nodular adrenocortical hyperplasia. Post-operatively, she successfully lost 20 kg in 18 months, with improvement in lipid profile, blood pressure and bone density at the spine. ACTH and DHEAS, which were previously suppressed, normalised.

611f7b67519a0-Figure+3a.jpg 

Autonomous cortisol secretion (ACS) is characterised by ACTH-independent cortisol overproduction from adrenal incidentalomas without clinical features of overt Cushing's syndrome. It is associated with metabolic consequences of obesity, hyperlipidaemia, hypertension, osteoporosis and increased cardiovascular mortality. ACS is more common in bilateral adrenal adenomas compared to unilateral lesions. Diagnosis and management are challenging due to controversies and discrepancies in current clinical guidelines and lack of long-term outcome data. Individualised clinical and risk assessment is important particularly in those with significant change in clinical features. The use of the 1 mg dexamethasone suppression test demonstrates the highest sensitivity for screening for ACS, with low/suppressed ACTH and DHEAS as useful adjuncts in the diagnosis.

  1. Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, Tabarin A, Terzolo M, Tsagarakis S, Dekkers OM. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175(2):1-34.
  2. Di Dalmazi G, Vicennati V, Garelli S, Casadio E, Rinaldi E, Giampalma E, Mosconi C, Golfieri R, Paccapelo A, Pagotto U, Pasquali R. Cardiovascular events and mortality in patients with adrenal incidentalomas that are either non-secreting or associated with intermediate phenotype or subclinical Cushing's syndrome: a 15-year retrospective study. Lancet Diabetes Endocrinol. 2014 May;2(5):396-405.
  3. Chiodini I, Albani A, Ambrogio AG, Campo M, De Martino MC, Marcelli G, Morelli V, Zampetti B, Colao A, Pivonello R; ABC Group. Six controversial issues on subclinical Cushing's syndrome. Endocrine. 2017;56(2):262-266
  4. Nieman LK. Update on subclinical Cushing's syndrome. Curr Opin Endocrinol Diabetes Obes. 2015;22(3):180-184
  5. Paschou SA, Kandaraki E, Dimitropoulou F, Goulis DG, Vryonidou A. Subclinical Cushing's syndrome in patients with bilateral compared to unilateral adrenal incidentalomas: a systematic review and meta-analysis. Endocrine. 2016;51(2):225-235