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Updated: Jun 11, 2025
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Reconstruct Human Retinoblastoma In Vitro
Published on: October 11, 2022
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Treatment Outcomes and Definition Inconsistencies in High-Risk Unilateral Retinoblastoma
Mattan Arazi1, Alona Baum2, Sandro Casavilca-Zambrano3
1From the Sheba Medical Center, The Goldschleger Eye Institute (M.A., I.D.), Sheba Medical Center, Ramat Gan, Israel; Faculty of Medicine, Tel Aviv University (M.A., A.B., I.D.), Tel Aviv, Israel; London School of Hygiene & Tropical Medicine (I.D., M. A.), London, UK.
American Journal of Ophthalmology
|September 27, 2024
Summary
Adjuvant chemotherapy after enucleation for retinoblastoma (Rb) with high-risk features significantly improves outcomes. Standardizing high-risk feature definitions globally is crucial to prevent adverse patient events.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Cancer Research
Background:
- Unilateral retinoblastoma (Rb) with high-risk histopathology features (HRHF) requires effective treatment strategies.
- Upfront enucleation is a primary surgical intervention for Rb.
- Adjuvant chemotherapy's role in improving outcomes for Rb with HRHF needs further clarification.
Purpose of the Study:
- To compare clinical outcomes in children with unilateral Rb and HRHF after upfront enucleation.
- To evaluate the impact of adjuvant chemotherapy versus no chemotherapy.
- To investigate the discrepancy in HRHF definitions and its effect on patient outcomes.
Main Methods:
- Retrospective multinational cohort study involving 21 centers across 12 countries (2011-2020).
- Included unilateral Rb cases undergoing primary enucleation.
- Standardized HRHF definition: retrolaminar optic nerve invasion, massive choroidal invasion, scleral invasion, anterior-segment involvement, and/or combined nonmassive choroidal and prelaminar/laminar optic nerve invasion.
Main Results:
- 600 children included; 505 received adjuvant chemotherapy.
- Adjuvant chemotherapy group had significantly lower rates of orbital tumor recurrence, metastasis, and death (P ≤ .002).
- 10.5% of cases initially classified as non-HRHF met the standardized HRHF definition, with 9.5% experiencing recurrence, metastasis, or death.
Conclusions:
- Adjuvant chemotherapy is recommended following enucleation for retinoblastoma with HRHF.
- Significant global variation in HRHF definition exists, leading to adverse outcomes.
- Standardization of HRHF definitions is warranted to improve patient care and outcomes.
