ACTH-ing up: Severe psychiatric presentation of fulminant ectopic ACTH syndrome and the role of bilateral adrenalectomy in the era of osilodrostat — ASN Events
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ACTH-ing up: Severe psychiatric presentation of fulminant ectopic ACTH syndrome and the role of bilateral adrenalectomy in the era of osilodrostat (#440)

Bridget Marshall 1 , Lyndal Tacon 1 2 , Sarina Lim 3
  1. Royal North Shore Hospital, St Leonards, NEW SOUTH WALES, Australia
  2. Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia
  3. Hornsby Ku-ring-gai Hospital, Sydney, NSW, Australia

With evolutions in surgical technique and new steroidogenesis inhibiting medications such as osilodrostat, the requirement of bilateral adrenalectomy for management of Cushing's syndrome is rare. There are however still patients for whom this may be required in the event of life-threatening hypercortisolism.

A previously well 18-year-old female presented following a high‑lethality suicide attempt in the context of a rapidly evolving depressive illness marked by escalating anxiety, intrusive thoughts, and tremulousness. She had no prior psychiatric, medical, or substance‑use history. In the Emergency Department, features of facial rounding and disproportionate dorsocervical and supraclavicular adiposity despite a BMI of 20.1 kg/m² were noted. Severe hypokalaemia (2.4 mmol/L) and markedly elevated evening cortisol (2389 nmol/L) prompted urgent endocrine evaluation. Investigations demonstrated; markedly elevated urinary free cortisol (3463 nmol/24 hr), elevated ACTH (71.4 pmol/L), and failure of suppression on low and high‑dose dexamethasone testing. Imaging demonstrated no pituitary lesion on MRI, adrenal hypertrophy on CT and an avid pancreatic head/neck lesion and multiple hepatic lesions on DOTATATE-PET. Osilodrostat was initiated but failed to control cortisol despite rapid uptitration. Her psychiatric state deteriorated despite intensive pharmacotherapy, with progressive flattening of mood and affect. In parallel, complications of severe hypercortisolism rapidly evolved, including refractory hypokalaemia, dyspepsia progressing to recurrent gastrointestinal haemorrhage, and profound proximal myopathy resulting in bedbound status within two weeks. Her treatment period was further complicated by QTc prolongation, and carcinoid crisis following liver biopsy. Given life‑threatening cortisol excess, the multidisciplinary team proceeded with bilateral adrenalectomy on day 24 of her admission for rapid control.

This case highlights how whilst rare, Cushing's syndrome can present as severe psychiatric disturbance. Furthermore with life-threatening hypercortisolism, complications of cortisol excess can accumulate faster than control can be achieved with medical therapy. It is in these cases where bilateral adrenalectomy for rapid cortisol control retains a role.