A pediatric patient with Cushing syndrome caused by ectopic ACTH syndrome and concomitant pituitary incidentalomas.
Ectopic ACTH syndrome is a rare but important cause of pediatric Cushing syndrome, for which management by a multidisciplinary team is required. Although diagnostic evaluation is similar to that in adults, the variation in epidemiology may sway investigations, leading to inappropriate and/or incomplete diagnostic interventions. We present a case of 15-year-old girl with symptoms of severe ACTH-dependent Cushing syndrome and two pituitary adenomas. The ectopic source of ACTH production was confirmed after petrosal venous sampling was performed. Diagnostics and perioperative management of a pulmonary carcinoid tumor producing ectopic ACTH is reviewed. In pediatric patients, as in adult patients, a pituitary lesion <6 mm on MRI is not sufficient confirmation of Cushing's disease, and appropriate diagnostic work-up should be performed to assess the source of the ACTH overproduction.
Alyse S
AS
Goldberg
Robert
R
Stein
Neil H
NH
Merritt
Richard
R
Inculet
Stan
S
Van Uum
D000182:ACTH Syndrome, Ectopic / Q000150:complications*; D000293:Adolescent; D002276:Carcinoid Tumor / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006801:Humans; D033162:Incidental Findings; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D010911:Pituitary Neoplasms / Q000150:complications / Q000175:diagnosis / Q000556:secondary*