Abstract
Purpose :
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.
Methods :
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.
Results :
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.
Conclusions :
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.