Background:
Cushing disease (CD) is a state of hypercortisolism caused by an adrenocorticotropic hormone-(ACTH) producing pituitary adenoma which rarely occurs in pediatric patients. The outstanding features are weight gain and growth retardation. However, the insidious onset and rarity of the disease in children and adolescents often result in delayed diagnosis.
Case description:
We present five patients <14 years of age who underwent neurosurgical treatment for CD at the Department of Neurosurgery of a public referral hospital in Lima, Peru. Age at diagnosis ranged from 5.5 to 12.5 years with a history of disease from 9 months to 3.5 years of moderate to severe stunting and obesity, among other features of Cushing syndrome (CS). Although biochemical tests and cerebral imaging were crucial for the diagnosis, confirmation was made by bilateral petrosal sinuous sampling. Regarding treatment, three patients underwent transcranial surgery, one patient underwent endoscopic transsphenoidal surgery, and one patient underwent microscopic transsphenoidal surgery. None of the patients underwent radiotherapy or pharmacological treatment. Only one patient had a recurrence and achieved remission until an endoscopic transsphenoidal approach was performed. Short- and long-term endocrinologic follow-up is also described in detail.
Conclusion:
CD is a heterogeneous disorder that requires multidisciplinary diagnosis and management. Transsphenoidal selective adenomectomy is the optimal treatment because of its higher remission rates. However, technical and anatomic aspects should be considered in pediatric patients.