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Preoperative diagnostic evaluation of children with Cushing's syndrome
S W Bickler1, T J McMahon, J R Campbell
1Department of Surgery, School of Medicine, Oregon Health Sciences University, Portland 97201.
Insights
Diagnosing Cushing's syndrome in children involves various tests to identify causes like adrenal tumors or pituitary adenomas. This study outlines a diagnostic strategy for pediatric hypercortisolism.
Area of Science:
- Pediatric Endocrinology
- Endocrinology
- Oncology
Background:
- Cushing's syndrome is rare in children, necessitating refined diagnostic approaches.
- Advances in biochemical and imaging techniques enhance diagnostic accuracy for hypercortisolism.
Purpose of the Study:
- To define the roles of diagnostic studies in pediatric Cushing's syndrome.
- To present a diagnostic strategy for noniatrogenic Cushing's syndrome in children.
Main Methods:
- Retrospective review of 15 pediatric patients with noniatrogenic Cushing's syndrome over 33 years.
- Utilized 19 different diagnostic tests reflecting evolving evaluation methods.
- Pathological diagnoses included adrenal cortical carcinoma, primary adrenocortical nodular dysplasia (PAND), and pituitary adenoma.
Main Results:
- Adrenal cortical carcinoma presented with an adrenal mass in younger children (mean age 22.3 months).
- PAND cases showed low plasma adrenocorticotrophin hormone (ACTH) and lack of suppression with high-dose dexamethasone.
- Pituitary adenomas occurred in older children (mean age 15.7 years), diagnosed via dexamethasone testing, inferior petrosal sinus sampling, and/or ovine corticotrophin-releasing hormone stimulation tests.
Conclusions:
- Differentiated diagnostic features exist for various causes of pediatric Cushing's syndrome.
- A strategic approach aids in diagnosing the underlying cause of hypercortisolism in children.
- Early identification and accurate diagnosis are crucial for effective management of pediatric Cushing's syndrome.
Abstract:
Recent advances in biochemical and imaging studies have improved the diagnostic accuracy of Cushing's syndrome. To better define roles for these studies in children, the authors reviewed their experience with this rare group of patients. Fifteen children, aged 11 weeks to 17 years, were treated for noniatrogenic Cushing's syndrome over a 33-year period. All children presented with signs of hypercortisolism. Nineteen different diagnostic tests were used, reflecting changes in how these patients are evaluated. Pathological diagnoses were adrenal cortical carcinoma (3), primary adrenocortical nodular dysplasia (PAND) (2), and pituitary adenoma (10). Children with adrenal cortical carcinoma presented with an adrenal mass and at a younger age (mean, 22.3 months). Key diagnostic features of patients with PAND were a low plasma adrenocorticotrophin hormone (ACTH) and no suppression with high-dose dexamethasone. Children with a pituitary cause of Cushing's syndrome presented at an older age (mean, 15.7 years) and were diagnosed using a combination of high-dose dexamethasone testing, simultaneous inferior petrosal sinus sampling, and/or ovine corticotrophin-releasing hormone stimulation test. A strategy for the diagnosis of Cushing's syndrome in children is presented.