title: Cushing syndrome associated with an adrenal tumour.
abstract: Cushing syndrome (CS) in children is a rare disorder that is most frequently caused by an adrenal tumour or a pituitary corticotrophin-secreting adenoma. The management is challenging and requires an individualised approach and multidisciplinary care. We present the case of a 23-month-old female child with a history of excessive weight gain, growth failure, hirsutism, acne and behavioural difficulties. Investigations revealed elevated serum midnight cortisol and 24&emsp14;h urinary free cortisol. Overnight dexamethasone suppression testing showed no suppression of cortisol levels. Abdominal imaging revealed a right-sided suprarenal mass. She underwent right adrenalectomy and the histology showed an adrenal cortical carcinoma. There was clinical improvement with catch-up growth and weight normalisation. Despite being rare in clinical practice, in a child with weight gain, hirsuitism and growth failure the diagnosis must be considered. The overall prognosis of CS in childhood is good, but challenges remain to ensure normal growth and body composition.
authors.affiliation: Paediatric Department, Hospital de São Francisco Xavier, Lisbon, Portugal. hrvieira@gmail.com
authors.forename: Helena
authors.initials: H
authors.lastname: Vieira
authors.forename: Caroline
authors.initials: C
authors.lastname: Brain
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000601:surgery; D001823:Body Composition / Q000502:physiology; D001827:Body Height / Q000502:physiology; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000601:surgery; D003907:Dexamethasone; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007223:Infant; D011379:Prognosis
chemical_list: D003907:Dexamethasone; D006854:Hydrocortisone