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Defining High-risk Retinoblastoma: A Multicenter Global Survey
Swathi Kaliki1, Carol L Shields2, Nathalie Cassoux3
1Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute India, Hyderabad, India.
JAMA Ophthalmology
|November 11, 2021
Summary
Definitions of high-risk retinoblastoma features lack global consensus, impacting metastasis risk assessment. Only optic nerve infiltration and extrascleral extension were universally recognized, highlighting the need for standardized criteria.
Area of Science:
- Ophthalmology
- Oncology
- Pathology
Background:
- High-risk histopathologic features in retinoblastoma are crucial for predicting systemic metastasis.
- The evolving landscape of globe salvage treatments necessitates a re-evaluation of these definitions.
Purpose of the Study:
- To investigate global variations in the definition of high-risk histopathologic features for retinoblastoma metastasis.
- To identify areas of consensus and divergence among international ocular oncology practices.
Main Methods:
- An anonymous, 10-question electronic survey was distributed to 52 international retinoblastoma specialists.
- The survey focused on identifying histopathologic features considered high-risk for metastasis.
Main Results:
- Twenty-seven specialists from 24 centers across 16 countries responded (52% response rate).
- Postlaminar optic nerve infiltration, optic nerve transection, and extrascleral tissue infiltration were universally identified as high-risk features (100%).
- Significant variability existed in the recognition of other features, including choroidal invasion, scleral infiltration, and anterior chamber seeding.
Conclusions:
- There is a notable lack of uniformity in defining high-risk retinoblastoma features globally.
- Standardized definitions and prospective multicenter studies are needed to clarify the prognostic value of these features in the context of modern, conservative treatment approaches.
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