Pediatric Adrenocortical Neoplasms: A Study Comparing Three Histopathological Scoring Systems

Area of Science:

• Pediatric Oncology
• Pathology
• Tumor Biology

Background:

• Adrenocortical neoplasms are rare in children, and their classification is challenging.
• Current prognostic tools like the AFIP/Wieneke system have limitations in pediatric cases.
• Newer scoring systems validated in adults require assessment in pediatric populations.

Purpose of the Study:

• To evaluate the applicability of the AFIP/Wieneke criteria, Helsinki score, and reticulin algorithm for predicting clinical outcomes in pediatric adrenocortical neoplasms.
• To compare the predictive accuracy of these histopathological scoring systems and the Ki-67 index.

Main Methods:

• Retrospective analysis of a cohort of pediatric adrenocortical neoplasms.
• Assessment of AFIP/Wieneke criteria, Helsinki score, and reticulin algorithm performance.
• Receiver Operating Characteristic (ROC) curve analysis and survival analysis (event-free and overall survival).
• Evaluation of Ki-67 index at a cut-off of 18%.

Main Results:

• The Helsinki score (cut-off 24) and reticulin algorithm demonstrated significant prognostic value, stratifying patients into distinct survival groups.
• The reticulin algorithm achieved 100% sensitivity, while the Helsinki score showed higher specificity (81.25%) than the reticulin algorithm (68.75%).
• The Ki-67 index (cut-off 18%) exhibited 80% sensitivity and 81.25% specificity for predicting unfavorable outcomes.

Conclusions:

• The Helsinki score and reticulin algorithm are valuable tools for predicting clinical outcomes in pediatric adrenocortical neoplasms.
• These newer systems offer improved prognostic accuracy compared to the AFIP/Wieneke criteria.
• The Ki-67 index also serves as a useful biomarker for risk stratification in these rare pediatric tumors.