Pediatric cyclical Cushing’s disease: a case report and literature review

Abstract

The article presents a rare clinical case of cyclical Cushing’s disease in a child. A 9‑year‑old boy complained of excessive weight gain for 3 years. Obesity had been progressing for the following 2 years, and then the child lost 14 kg in 5 months. Growth hormone deficiency with regard to slow growth rate and hypogonadism due to maintaining of prepubertal gonadal size were excluded. Taking into account the history of obesity and low growth rate, the child was repeatedly examined for hypercortisolism. The urinary 24‑h cortisol excretion and the overnight dexamethasone suppression test didn’t confirm hypercortisolism in spite of detected impairment of the adrenocorticotropic hormone circadian rhythm. Resumption of weight gain was observed at the age of 13.5 years. The results of urinary 24‑h cortisol excretion confirmed hypercortisolism. Magnetic resonance imaging of pituitary was unremarkable. The boy underwent simultaneous bilateral selective blood sampling from the cavernous, inferior petrosal, and sigmoid sinuses. Significant positive adrenocorticotropic hormone gradient was registered in the left cavernous sinus compared to peripheral levels, so Cushing’s disease was diagnosed. Endoscopic transnasal surgery was conducted and abnormal tissue was resected. Histopathology revealed no pituitary adenoma. The article provides a brief review on the cyclic Cushing’s disease in children and the main reasons of negative results of histopathology.