Introduction: Cushings disease (CD) is the most common albeit rare in paediatric and adolescent population form of ACTH-dependent Cushings syndrome, with potentially serious morbidity. Thus, it presents diagnostic and therapeutic challenges for the clinician. Early diagnosis and treatment of Cushings disease is vital for long-term outcome. Paediatric pituitary-dependent Cushings disease, caused by an ACTH-secreting corticotroph adenoma, accounts for 7580% of Cushings syndrome and is almost always caused by a pituitary microadenoma.
Case report: 14 yo girl presented to endocrine clinic with severe headaches, increased blood pressure (BP) up to 140/100 mmHg, wide reddish-purple abdominal striae, amenorrhea and weight gain up to 20 kg during last year. She was unsuccessfully managed by a gynecologist with oral contraceptives for 8 months. In endocrine clinic patient was examined according to Endocrine Society guidelines with subsequent diagnosis of Cushings disease due to ACTH-secreting pituitary microadenoma (corticotropinoma). Patient underwent surgical removal of adenoma with gamma knife (GKS) resulting in reversal of symptoms.
Conclusions and follow up: Endocrine parameter normalization after GKS included normal 24-hour urinary free cortisol (UFC) concentration and normal levels of pituitary and peripheric hormones 4 months. However, posttreatment secondary hypothyroidism was diagnosed with TSH of 2.2 IU/ml (0.54.0) and FT4 of 12.1 pmol/l (12.022.0). BP was stable at 110/70 mmHg. Regular menstrual function resumed 6 months after the surgery, and weight loss of 10 kg was documented 10 months after the surgery. Adenoma size decreased from 0.6 to 0.2 cm in 7 months by MRI. Current medications comprised of Levothyroxin 75 mcg only. This is the first case of successful treatment of paediatric Cushings disease in Armenia. Although transsphenoidal neurosurgery is the gold standard therapy of pituitary Cushings disease, GKS seems to be safe and effective way of treatment, however, long-term follow-up is necessary.
20 - 23 May 2017
European Society of Endocrinology