Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?

Cushing’s disease (CD) is the most common cause of endogenous Cushing’s syndrome in children and adolescents and represents a rare cause of short stature. A 14-year-old boy came to our attention for progressive weight gain and short stature. At examination, height was 140 cm (3rd centile) and weight...

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Main Authors: Rosa Maria Paragliola, Pietro Locantore, Alfredo Pontecorvi, Salvatore Maria Corsello
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Endocrinology
Online Access:http://dx.doi.org/10.1155/2014/851942
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author Rosa Maria Paragliola
Pietro Locantore
Alfredo Pontecorvi
Salvatore Maria Corsello
author_facet Rosa Maria Paragliola
Pietro Locantore
Alfredo Pontecorvi
Salvatore Maria Corsello
author_sort Rosa Maria Paragliola
collection DOAJ
description Cushing’s disease (CD) is the most common cause of endogenous Cushing’s syndrome in children and adolescents and represents a rare cause of short stature. A 14-year-old boy came to our attention for progressive weight gain and short stature. At examination, height was 140 cm (3rd centile) and weight was 37.7 kg (10th centile). Tanner stage was G2, PH 3, testis 3 mL. Hypothyroidism and growth hormone deficiency were excluded. A marked increase of urinary free cortisol, a nonsuppressible serum cortisol after Liddle 1 test, and an elevated ACTH value confirmed the diagnosis of ACTH dependent Cushing’s syndrome. Pituitary magnetic resonance imaging (MRI) showed a left microadenoma and a right focal area of lesser enhancement. Therefore, bilateral inferior petrosal sinus sampling (BIPSS) with CRH stimulation was performed to obtain an accurate preoperative localization of the adenoma: the interpetrosal sinus ACTH gradient indicated lateralization of ACTH secretion to the left side. The patient underwent transsphenoidal surgery with selective microadenomectomy, with an immediate ACTH decline in the postoperative phase. Histology confirmed the diagnosis of corticotrophic pituitary adenoma. Glucocorticoid replacement therapy was instituted. Clinical examination demonstrated a rapid catch-up growth (10th centile), with a normalization of body mass index and an adequate pubertal development.
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spelling doaj-art-efd21df2e1ea476b84cdeb6cd579f8f82025-08-20T03:21:22ZengWileyCase Reports in Endocrinology2090-65012090-651X2014-01-01201410.1155/2014/851942851942Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?Rosa Maria Paragliola0Pietro Locantore1Alfredo Pontecorvi2Salvatore Maria Corsello3Endocrinology Unit, Catholic University School of Medicine, Largo Gemelli No. 8, 00168 Rome, ItalyEndocrinology Unit, Catholic University School of Medicine, Largo Gemelli No. 8, 00168 Rome, ItalyEndocrinology Unit, Catholic University School of Medicine, Largo Gemelli No. 8, 00168 Rome, ItalyEndocrinology Unit, Catholic University School of Medicine, Largo Gemelli No. 8, 00168 Rome, ItalyCushing’s disease (CD) is the most common cause of endogenous Cushing’s syndrome in children and adolescents and represents a rare cause of short stature. A 14-year-old boy came to our attention for progressive weight gain and short stature. At examination, height was 140 cm (3rd centile) and weight was 37.7 kg (10th centile). Tanner stage was G2, PH 3, testis 3 mL. Hypothyroidism and growth hormone deficiency were excluded. A marked increase of urinary free cortisol, a nonsuppressible serum cortisol after Liddle 1 test, and an elevated ACTH value confirmed the diagnosis of ACTH dependent Cushing’s syndrome. Pituitary magnetic resonance imaging (MRI) showed a left microadenoma and a right focal area of lesser enhancement. Therefore, bilateral inferior petrosal sinus sampling (BIPSS) with CRH stimulation was performed to obtain an accurate preoperative localization of the adenoma: the interpetrosal sinus ACTH gradient indicated lateralization of ACTH secretion to the left side. The patient underwent transsphenoidal surgery with selective microadenomectomy, with an immediate ACTH decline in the postoperative phase. Histology confirmed the diagnosis of corticotrophic pituitary adenoma. Glucocorticoid replacement therapy was instituted. Clinical examination demonstrated a rapid catch-up growth (10th centile), with a normalization of body mass index and an adequate pubertal development.http://dx.doi.org/10.1155/2014/851942
spellingShingle Rosa Maria Paragliola
Pietro Locantore
Alfredo Pontecorvi
Salvatore Maria Corsello
Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
Case Reports in Endocrinology
title Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
title_full Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
title_fullStr Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
title_full_unstemmed Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
title_short Pediatric Cushing’s Disease and Pituitary Incidentaloma: Is This a Real Challenge?
title_sort pediatric cushing s disease and pituitary incidentaloma is this a real challenge
url http://dx.doi.org/10.1155/2014/851942
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