Risk Factors for Extraocular Relapse in Retinoblastoma.

Abstract

Background: Metastatic retinoblastoma remains a significant challenge in pediatric oncology, with stark disparities in survival outcomes between high-income countries (HICs) and low-income countries (LICs). Delayed diagnosis and treatment, driven by socioeconomic factors and limitations in healthcare systems, contribute to poorer outcomes in LICs. Histopathological characteristics, including high-risk pathology factors (HRPFs) and the extent of ocular tumor invasion, are critical for predicting metastatic risk and guiding treatment strategies.

Methods: This review examines the role of clinical, histopathological, and molecular characteristics in assessing metastatic risk in retinoblastoma. Literature on HRPFs, tumor invasion, and molecular subtypes was analyzed to understand their impact on risk stratification and therapy optimization, particularly in resource-limited settings.

Results: Retinoblastoma is increasingly recognized as a heterogeneous disease with at least two distinct molecular subtypes. High-risk cases frequently exhibit genetic alterations that underscore the need to incorporate molecular profiling into risk assessment. Current adjuvant therapy approaches, however, vary widely, and debates persist regarding their necessity based on tumor characteristics. Integrated strategies that combine clinical, histopathological, and molecular data show promise in improving management and survival outcomes.

Conclusions: Addressing the disparities in metastatic retinoblastoma outcomes requires a multifaceted approach. By integrating clinical, histopathological, and molecular insights, management strategies can be optimized to improve survival, particularly in resource-limited settings where challenges are most pronounced.