Literature DB >> 22927284

Cushing syndrome associated with an adrenal tumour.

Helena Vieira1, Caroline Brain.   

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 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.

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Year:  2012        PMID: 22927284      PMCID: PMC4543960          DOI: 10.1136/bcr-2012-006685

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  4 in total

Review 1.  Work-up and management of paediatric Cushing's syndrome.

Authors:  Martin O Savage; Li F Chan; Ashley B Grossman; Helen L Storr
Journal:  Curr Opin Endocrinol Diabetes Obes       Date:  2008-08       Impact factor: 3.243

2.  Childhood Cushing disease: a challenge in diagnosis and management.

Authors:  Nalini S Shah; Anurag Lila
Journal:  Horm Res Paediatr       Date:  2011-07-21       Impact factor: 2.852

3.  Outcome of adrenocortical tumors in children.

Authors:  Angela M Hanna; Tuan H Pham; Johanna R Askegard-Giesmann; Jayleen M Grams; Corey W Iqbal; Penny Stavlo; Christopher R Moir
Journal:  J Pediatr Surg       Date:  2008-05       Impact factor: 2.545

4.  Diagnostic tests for children who are referred for the investigation of Cushing syndrome.

Authors:  Dalia L Batista; Jehan Riar; Meg Keil; Constantine A Stratakis
Journal:  Pediatrics       Date:  2007-08-13       Impact factor: 7.124

  4 in total

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