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Christina Scelfo, Jasmine H Francis, Thomas Lee Jenkins, Brian P Marr, David H Abramson; An International Survey of Classification and Treatment Choices for Group D Retinoblastoma Eyes. Invest. Ophthalmol. Vis. Sci. 2016;57(12):3676.
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© ARVO (1962-2015); The Authors (2016-present)
The purpose of this international survey of Group D retinoblastoma was to: 1) determine which International Classification of Retinoblastoma (ICRB), scheme was used by retinoblastoma centers worldwide, 2) determine the percentage of D eyes primarily enucleated in each center, 3) correlate initial treatment options and patient outcome and 4) report on patient survival worldwide.
An anonymized electronic survey was offered to 115 physicians at 39 retinoblastoma centers worldwide asking about treatment patterns and classification schemes used between 2008-2012.
The survey was completed by 29.5% of centers dispersed through Asia, Africa, Middle East, Europe, US, and Latin America. 1807 D eyes were diagnosed. Regarding ICRB system, 27.6% of centers used the Children’s Hospital of Los Angeles version, 34.5% used the Children's Oncology Group version, 24.1% used the Philadelphia version, and 13.8% were unsure which version they used. The rate for primary enucleation varied between 0 and 100%. By continent, the primary enucleation rates were: South America, 57.7%; Asia, 40.4%; Europe, 36.0%; US, 8.4%; and Middle East, 8.3% as shown in Figure 1. 26% of centers did not primarily enucleate any eye and 26% of centers primarily enucleated greater than 50% of eyes. Patient survival ranged from 0% to 100% following primary enucleation and 31.2% to 100% following globe-salvaging strategies, with summative data demonstrated in Figure 2.
The above results suggest there is no consensus on which ICRB version is used to classify group D eyes worldwide. Regarding treatment, the primary enucleation rate varied greatly across centers, particularly by continent. Systemic chemoreduction, however, was the most frequently used initial treatment for group D eyes treated between 2008 and 2012. Survival was greatest in patients treated with either bridging chemotherapy plus intra-arterial chemotherapy followed by intra-arterial chemotherapy alone.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
Figure 1. AFR=Africa, EUR=Europe, ME=Middle East, LA=Latin America.
Figure 2. *Based on mortality from metastatic disease, complications of treatment, or secondary cancer.
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