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Adrenocortical tumors in children
R C Ribeiro1, E L Michalkiewicz, B C Figueiredo
1Department of Hematology-Oncology, College of Medicine, University of Tennessee, Memphis, TN 38105, USA. raul.ribeiro@stjude.org
Summary
Childhood adrenocortical tumors (ACT) are rare globally but highly prevalent in Southern Brazil. Diagnosis relies on hormone levels and imaging, with surgery being key, though prognosis varies by tumor size and spread.
Area of Science:
- Pediatric Endocrinology
- Oncology
- Genetics
Background:
- Childhood adrenocortical tumors (ACT) are rare in the USA, with ~25 annual cases.
- Southern Brazil exhibits a significantly higher incidence, approximately 10 times that of the USA.
- The etiology of this regional disparity remains unidentified, with no clear genetic links to known ACT predispositions.
Purpose of the Study:
- To summarize the epidemiology, clinical presentation, diagnosis, pathology, treatment, and prognosis of childhood adrenocortical tumors (ACT).
- To highlight the unique epidemiological characteristics of ACT in Southern Brazil.
- To discuss diagnostic challenges and prognostic factors in pediatric ACT.
Main Methods:
- Review of clinical data, diagnostic markers (urinary 17-ketosteroids, plasma DHEA-S), imaging findings, pathological classification, and treatment outcomes.
- Analysis of prognostic indicators including tumor resection status, metastatic disease, and tumor size.
Main Results:
- Affected children are typically young girls with virilizing and/or Cushing syndromes.
- Abnormal hormone levels (90% of cases) and characteristic imaging (calcifications, necrosis) aid diagnosis.
- Tumor size predicts relapse risk in patients with localized, resected tumors.
Conclusions:
- Surgery is the primary treatment for pediatric ACT.
- Chemotherapy's role is unestablished, though some tumors respond to specific regimens.
- Prognosis is poor for metastatic or incompletely resected tumors; smaller tumors correlate with better outcomes.