Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2006) 11 P438

ECE2006 Poster Presentations Endocrine tumours and neoplasia (116 abstracts)

Challenges associated with the diagnosis, differential diagnosis and treatment of 4 cases of ACTH-dependent Cushing’s syndrome due to intermittent hypercortisolism

JR Lindsay & LK Nieman


Reproductive Biology and Medicine Branch, NICHD, National Institutes of Health, Bethesda, MD, United States.


Episodic hypercortisolism has been widely described in Cushing’s disease (CD) but is a less commonly recognized presentation of ectopic ACTH syndrome (EAS). We describe 4 cases whose diagnosis and management was complicated by episodic hypercortisolism. These cases highlight diagnostic and therapeutic challenges during the differential diagnosis of CS complicated by intermittent hypercortisolism. All 3 cases of EAS required various combinations of ketoconazole, metyrapone and aminoglutethimide as primary medical therapy for control of hypercortisolism. While EAS is a less common cause of cyclic ACTH-dependent CS, it should be considered in cases with extreme hypercortisolism or hypokalemia. In cases with occult EAS, bilateral adrenalectomy (BAD) may be required due to difficulties in safely achieving long-term eucortisolism with medical therapy.

Table 1
Cases (Age, Y; Gender)
64 M Occult EAS53 F EAS52 F Occult EAS52 M CD
UFC (interval; days) nmol/d202–6889 (19)58–7080 (568)*80–5824 (270)*72–173 (6)*
ImagingPersistently negative1.5 cm pancreatic tumorLesion: mediastinumN/A
Surgery (ACTH staining)BADDistal pancreatectomy (+)Thoracotomy (−); BADTSS (+)
ChallengeDifferential DiagnosisEpisodic hypercortisolismExtreme hypo/hypercortisolismDiagnosis
*Pre-treatment; On ketoconazole

Volume 11

8th European Congress of Endocrinology incorporating the British Endocrine Societies

European Society of Endocrinology 
British Endocrine Societies 

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