ECE2020 Audio ePoster Presentations Pituitary and Neuroendocrinology (217 abstracts)
Hospital Universitario Miguel Servet, Zaragoza, Spain
Introduction: Cyclic Cushing´s syndrome (CCS) is a rare disorder with a wide clinical variability, characterized by repeated episodes of cortisol excess and periods of normal or even low cortisol secretion. Different periodical diagnostic tests are required to confirm hypercortisolism and thereafter imaging techniques and laboratory studies can be performed to localize the cause.
Case report: A 17-year-old female patient with a personal history of advanced puberty and non-treated obesity is admitted to Internal Medicine reporting myalgias, headache and a rapid weight gain of 15 kgs. Physical examination reveals facial plethora, violaceous striae, hirsutism and menstrual disorders. In conjunction with Endocrinology, initial laboratory testing is guided towards diagnosis of Cushing syndrome, but all results result normal. However, due to a great clinical suspicion, the study is prolonged and during the follow-up, alternating normal and abnormal tests are obtained. Petrosal sinus sampling is indicated concurring with abnormal 1 mg- dexamethasone suppression and pituitary magnetic resonance showed a 4 mm adenoma.
08/02/19 | 01/03/19 | 20/03/19 | 08/04/19 | ||||
24 h free cortisol µg/24 hours | 43.86 | 36.46 | 279.55 | 44.53 | |||
Nugent µg/dl | 0.84 | 2.43 | 11.67 | ||||
Cortisol 00:00 h µg/dl | 0.88 | 7.04 | 8.17 | 5.95 | |||
Cortisol 8:00 h µg/dl | 2.94 | 9.80 | |||||
Cortisol 20:00 h µg/dl | 1.31 | 3.93 | |||||
ACTH pg/ml | 23.1 | 37 | 28.8 | 21.5 | |||
Petrosal sinus sampling | |||||||
Peripheral | RPS | LPS | |||||
ACTH pg/ml | 34.6 | 478.8 | 92.13 | ||||
ACTH 5 minafter CRH pg/ml | 73.9 | 80.3 | 53.4 |
A successful hemi-hypophysectomy is performed with great clinical improvement and an ACTH-producing pituitary adenoma is reported in the pathology sample.
Discussion: Cycles of hypercortisolism in CSS can occur regularly or irregularly with intercyclic phases ranging from days to years showing normal cortisol or even hypocortisolemia. The fluctuating clinical picture and discrepant biochemical findings make cyclic CCS sometimes extremely hard to diagnose.
Ethical aspects: The case has the informed consent of the patient.