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Publication Date:
September 2010
ISSN:
2191-0251
DOI:
10.1515/jpem.2010.100

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Editor-in-Chief: Zadik, Zvi

Editorial Board Member: Cassorla, Fernando / Cutfield, Wayne / de Muinck Keizer-Schrama, Sabine M.P.F. / Fideleff, Hugo L. / LaFranch, Stephen H. / Lanes M. D., Roberto / Levitsky, Lynne / Lippe, Barbara / Pfäffle, Roland / Root, Allen W. / Rosenfeld, Ron G. / Werther, George / Kiess, Wieland

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An Unusual Presentation of Pediatric Cushing Disease: Recurrent Corticotropinoma of the Posterior Pituitary Lobe

Monalisa F. Azevedo1 / Paraskevi Xekouki1 / Meg F. Keil12 / Eileen Lange1 / Nicholas Patronas3 / Constantine A. Stratakis12

1Section 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

2Inter-Institute Pediatric Endocrinology Training Program, National Institutes of Health (NIH), Bethesda, MD 20892, USA

3Diagnostic Radiology, Clinical Center, National Institutes of Health (NIH), Bethesda, MD 20892, USA

c1Corresponding author: Dr. Monalisa Ferreira Azevedo,

Citation Information: Journal of Pediatric Endocrinology and Metabolism. Volume 23, Issue 6, Pages 607–612, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: 10.1515/jpem.2010.100, September 2010

Publication History:
Published Online:
2010-09-17

ABSTRACT

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.

KEY WORDS: Cushing disease; pituitary adenoma; microadenoma

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