ECE2017 Eposter Presentations: Pituitary and Neuroendocrinology Clinical case reports - Pituitary/Adrenal (41 abstracts)
Faculty of Medicine and Pharmacy, Marrakech, Morocco.
Introduction: Cyclic Cushings syndrome is a rare disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion ranging from days to years. It remains a difficult diagnostic challenge in endocrinology, we report a case.
A case report: The patient was 41 years old woman, she presented in 2012 a period of symptomatic hypercortisolism. Urinary free cortisol was elevated to 113 μg/24, Low-dose dexamethasone testing was negative, ACTH was elevated to 20.2 pmol/l, the high-dose dexamethasone suppression test was positive, a pituitary MRI and a thoraco pancreatic scanner were normal; These finding led us to an ACTH-dependent Cushings syndrome. The patient was revaluated 5 months later and she was clinically and biochemically better. Six months later she presented relapsed symptoms and a biochemical recurrence of hypercortisilosm (high urinary cortisol: 125.7 μg/24 and negative Low-dose dexamethasone testing). In 2015 the patient presented a sudden tumor syndrome, pituitary MRI revealed an intrasellar arachnoidocele that was in favour of the apoplexy of an adrenocorticotropic pytuitary adenoma. The patient was revaluated 9 months later and she was clinically better, The Urinary free cortisol was normal (20 mg/24).
Discussion: Cyclic Cushings syndrome is characterized by rhythmic fluctuations in glucocorticoid production, both clinical and biochemical spontaneous remissions may occur in patients with this disorder. Our patient presented two periods of normal glucocorticoid production and two periods of hypercortisolism. Causes of cyclic Cushings syndrome are multiple dominated by pituitary adenoma. The biological and radiological investigations in this case and therfore the subsequent evolution enabled a diagnosis of a Cushings syndrome spontaneously regressive by the apoplexy of pytiutary adrenocorticotropic adenoma. Clinicians should be aware that hypercortisolism may occur periodically and the duration of the periods of normal and abnormal cortisol secretion can vary significantly, so the correct diagnosis can be a challenge in clinical practice.