An unusual presentation of pediatric Cushing disease: recurrent corticotropinoma of the posterior pituitary lobe.
Cushing's syndrome (CS) is rare in childhood and adolescence and its diagnosis and work up are often challenging. We report the case of a 15-year-old girl with a recurrent corticotrophin (ACTH)-secreting adenoma, located in the posterior lobe of the pituitary gland. At the age of 11, she presented with classic CS symptoms; biochemical investigation was compatible with ACTH-dependent Cushing disease, although pituitary gland imaging did not show any tumor. Following transsphenoidal surgery (TSS), histopathological analysis identified an ACTH-secreting pituitary microadenoma arising from the posterior gland. The patient went into remission but 4 years later she presented with recurrent CS; this time, pituitary gland imaging showed a microadenoma located in the posterior lobe, which was resected after TSS. Posterior lobe pituitary adenomas are very rare and often hard to diagnose and treat; this is the first case of such a tumor causing recurrent Cushing's disease in a child.
Azevedo
Monalisa F
MF
Section on Endocrinology and Genetics, Program on Developmental Endocrinology Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD 20892, USA. monalisa.azevedo@nih.gov
Xekouki
Paraskevi
P
Keil
Meg F
MF
Lange
Eileen
E
Patronas
Nicholas
N
Stratakis
Constantine A
CA
D049913:ACTH-Secreting Pituitary Adenoma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D000236:Adenoma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009364:Neoplasm Recurrence, Local; D047748:Pituitary ACTH Hypersecretion / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery