ESPE Abstracts

Background: Cushing’s disease (CD) is caused by an ACTH-secreting pituitary corticotroph adenoma and it is the commonest cause of Cushing’s syndrome (CS) in pediatric age. CD is very rare disease almost in pediatric age and the most of pediatric endocrinologists have limited experience in the diagnosis and treatment of children with CD. Microadenomas are the most of ACTH-secreting pituitary tumors in the pediatric age and the majority of these fails to detect on MRI because they have a hypointense signal on this examination and they show a normal appearance in about half of the patients. Therefore, results should be interpreted together with bilateral inferior petrosal sinus sampling (BIPSS) for ACTH.

Case: A 12 years old boy was presented with headache, rapid weight gain during 3 years, stunned height, increase hair growth and a typical Cushingoid appearance. His investigations showed the following values: cortisol 26.8 mcg/dL, ACTH 95.3 pg/ml (08⁰⁰ a.m); cortisol 30.6 mcg/dL, ACTH 116.2 pg/ml (04⁰⁰ p.m); salivary cortisol 10.55 ng/ml and 25.9 ng/ml (1100 p.m. in two consecutive days); free cortisol level in 24-h urine 556 mcg/24-h; cortisol prior to high-dose dexamethasone suppression test (HDDST) was 13.8 mcg/dL and 12.7 mcg/dL after it and DHEA-s 894 mcg/dL. FDG-PET Scan was performed but no ectopic ACTH-dependent focus was detected. An intervention to collect samples from inferior petrosal sinus (BIPSS) was performed under general anesthesia. Findings were consistent with left sided ACTH-secreting focus (ACTH 139 pg/ml in the right petrosal sinus and >2000 pg/ml in the left petrosal sinus after CRH). The patient underwent gamma knife therapy and after that he started using low dose of ketoconazole for medical adrenalectomy to inhibit steroidogenesis.

Keywords: Cushing’s disease, Cushing’s syndrome, Pediatrics, bilateral inferior petrosal sinus sampling, gamma knife.