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Adrenocortical tumors in children.
A O Ciftci1, M E Senocak, F C Tanyel
1Department of Pediatric Surgery, Hacettepe University Medical Faculty, 06100, Ankara, Turkey.
Journal of Pediatric Surgery
|April 3, 2001
Summary
Pediatric adrenocortical tumors (ACTs) management is challenging. Early diagnosis and complete surgical excision are crucial for improving outcomes in adrenocortical carcinoma (ACC), while adrenocortical adenomas (ACAs) are successfully treated with surgery.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Surgical Oncology
Background:
- Pediatric adrenocortical tumors (ACTs) are rare, making their etiopathogenesis and management poorly understood.
- This study reviews a large single-center series of pediatric ACTs to provide a comprehensive overview.
Purpose of the Study:
- To delineate the full spectrum of pediatric adrenocortical tumors (ACTs).
- To analyze clinical characteristics, diagnostic methods, treatment, and outcomes for pediatric ACTs.
Main Methods:
- Retrospective review of 30 pediatric patients treated for ACTs between 1970 and 1999.
- Patients were classified into adrenocortical carcinoma (ACC) and adrenocortical adenoma (ACA) groups for comparative analysis.
Main Results:
- Of 30 children, 20 had ACC and 10 had ACA. Endocrine dysfunction (83%) was common, with virilization and Cushing's syndrome as primary presentations.
- ACC cases often presented with advanced disease (regional or metastatic), while ACAs were typically localized.
- Complete surgical excision was curative for ACA; however, for ACC, partial excision and advanced stage significantly correlated with poor outcomes.
Conclusions:
- Adrenocortical adenomas (ACAs) are effectively managed with complete surgical excision.
- Early diagnosis and complete resection are paramount for improving survival in pediatric adrenocortical carcinoma (ACC).
- Further multi-institutional trials are needed to establish optimal treatment protocols and understand the molecular mechanisms of ACTs.