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Childhood adrenocortical tumours
1The Department of Hematology-Oncology, St. Jude Children's Research Hospital, and Department of Pediatrics, University of Tennessee College of Medicine, Memphis, US. raul.ribeiro@stjude.org
European Journal of Cancer (Oxford, England : 1990)
|April 28, 2004
Summary
Childhood adrenocortical tumours (ACT) are rare paediatric cancers, with higher incidence in southern Brazil. Complete surgical resection offers the best prognosis for these virilizing tumours.
Area of Science:
- Pediatric Oncology
- Endocrinology
- Genetics
Background:
- Childhood adrenocortical tumours (ACT) are rare, accounting for 0.2% of pediatric malignancies.
- ACT incidence varies geographically, notably high in southern Brazil.
- Most pediatric ACT cases present with virilization due to hormone hypersecretion.
Purpose of the Study:
- To summarize the clinical presentation, diagnosis, and management of childhood adrenocortical tumours.
- To identify prognostic factors and treatment outcomes for pediatric ACT.
Main Methods:
- Review of clinical and pathological features of childhood ACT.
- Evaluation of diagnostic imaging modalities (CT, MRI).
- Analysis of treatment strategies including surgery, chemotherapy, and radiotherapy.
Main Results:
- Virilization is the most common presenting sign; some patients lack endocrine symptoms.
- P53 gene mutations are frequently associated with ACT.
- Complete tumor resection is crucial for cure; prognostic factors include age, tumor size, and histology.
Conclusions:
- Complete surgical resection is the cornerstone of ACT treatment.
- Favorable prognostic indicators aid in patient management and outcome prediction.
- Further research is needed to establish the roles of chemotherapy and radiotherapy.