Poster Presentation The Annual Scientific Meeting of the Endocrine Society of Australia and the Society for Reproductive Biology 2014

A case of severe ectopic ACTH syndrome from an occult primary – diagnostic and management dilemmas (#294)

Harish Venugopal , Katherine Griffin 1 , Saima Amer 1
  1. Department of Diabetes and Endocrinology, Gold Coast University Hospital, Gold Coast, Queensland, Australia

Case report:

We present the case of a 66-year old gentleman with Cushing’s syndrome with a 12-month history of rapidly declining mobility due to severe proximal muscle weakness and atrophy associated with multiple vertebral fractures, poorly-controlled diabetes and recurrent urinary tract infections. Diagnosis of ACTH-dependant Cushing’s syndrome was confirmed by non-suppression with overnight low-dose dexamethasone suppression test, elevated 24-hour urinary free cortisol and ACTH levels. Ectopic ACTH secretion was suggested by non-suppression with high-dose dexamethasone suppression test and bilateral inferior petrosal sinus sampling. CT scan of head, chest, abdomen and pelvis and a gallium-68 Dotatate-PET scan failed to identify the ectopic source.

Maximum tolerated doses of ketoconazole and metyrapone failed to control his hypercortisolaemia. Persisting severe hypercortisolism and immunocompromised state led to recurrent intra-abdominal and pelvic abscesses, requiring multiple surgical interventions.  He had also developed severe cardiac strain in the process making him subsequently unfit for any further surgeries. Laparoscopic bilateral adrenalectomy was considered, however decided against given concerns of technical difficulties due to recurrent intra-abdominal infections and his moribund state.  Alcohol ablation of adrenal glands by retrograde adrenal vein approach was subsequently attempted resulting in biochemical remission of Cushing’s syndrome. He, thereafter, improved clinically and underwent rehabilitation. 


Resection of the primary tumour is the management of choice in patients with ectopic ACTH syndrome1 . However, tumours may remain unidentified or occult in spite of extensive efforts at trying to locate them. This can therefore pose a major management issue as uncontrolled hypercortisolaemia can lead to lift-threatening infections2 . Bilateral adrenalectomy should be considered if adrenolytic medical treatment fails to lower cortisol levels adequately3 . However, patients may be extremely unwell and unfit to undergo such a procedure. Percutaneous interventions like retrograde adrenal vein alcohol ablation may be attempted in such patients and can induce biochemical remission4, 5  .

  1. Isidori AM, Kaltsas GA, Pozza C, Frajese V, Newell-Price J, Reznek RH, et al. The ectopic adrenocorticotrophin syndrome: clinical features, diagnosis, management and long-term follow-up. J Clin Endocrinol Metab. 2006;91:371–7
  2. Grossman AB, Kelly P, Rockall A, Bhattacharya S, McNicol A, Balwick T. Cushing’s syndrome caused by an occult source: difficulties in diagnosis and management. Nature Clinical Practice: Endocrinology and Metabolism. Nov 2006. Vol 2. No 11
  3. Salameh JR, Borman KR, Varkarakis GM. Laparoscopic bilateral adrenalectomy for occult ectopic ACTH syndrome. J Laparoendosc Adv Surg Tech A. 2008 Feb;18(1):52-5
  4. Venkatesan AM1, Locklin J, Dupuy DE, Wood BJ. Percutaneous ablation of adrenal tumors. Tech Vasc Interv Radiol. 2010 Jun;13(2):89-99
  5. Gatti A, Baroni MG, Romeo S, Mandosi E, Fallarino M, Bezzi M, Paciucci L, Filetti S, Morano S. A case of severe occult ectopic adrenocorticotropin syndrome treated with retrograde venous adrenal ablation using ethanol. J Endocrinol Invest. 2008 Dec;31(12):1135-6