July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Tumor size criteria for Group D and E eyes in the International Classification System for Retinoblastoma: effect on rates of globe salvage and high-risk histopathologic features
Author Affiliations & Notes
  • Sona Shah
    Keck School of Medicine of USC, Los Angeles, California, United States
    USC Roski Eye Institute, California, United States
  • Jonathan W. Kim
    USC Roski Eye Institute, California, United States
    Children's Hospital Los Angeles, California, United States
  • Sarah Green
    Children's Hospital Los Angeles, California, United States
  • John O'Fee
    Keck School of Medicine of USC, Los Angeles, California, United States
    USC Roski Eye Institute, California, United States
  • Benita Tamrazi
    Children's Hospital Los Angeles, California, United States
  • Jesse L Berry
    USC Roski Eye Institute, California, United States
    Children's Hospital Los Angeles, California, United States
  • Footnotes
    Commercial Relationships   Sona Shah, None; Jonathan Kim, None; Sarah Green, None; John O'Fee, None; Benita Tamrazi, None; Jesse Berry, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 2325. doi:https://doi.org/
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      Sona Shah, Jonathan W. Kim, Sarah Green, John O'Fee, Benita Tamrazi, Jesse L Berry; Tumor size criteria for Group D and E eyes in the International Classification System for Retinoblastoma: effect on rates of globe salvage and high-risk histopathologic features. Invest. Ophthalmol. Vis. Sci. 2019;60(9):2325. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : The International Classification System for Retinoblastoma has become the standard method for diagnosing intraocular retinoblastoma. However, there are currently discrepancies in some aspects of the system, particularly when classifying advanced cases (i.e. Groups D and E). The goal of this study was to analyze the impact of a large tumor size criteria for classifying advanced intraocular retinoblastoma on rates of globe salvage and high-risk histopathologic features.

Methods : A retrospective chart review was conducted of 212 eyes diagnosed with Group D (111 eyes) or Group E (101 eyes) retinoblastoma in at least one eye from January 1, 2006 to December 31, 2016 using the Los Angeles (LA) Classification System (no size criteria for Group E). The 111 Group D tumors were then reclassified to Group E using 10, 12, 14, 16, and 18 mm tumor size criteria as determined by ultrasound or MRI.

Results : For eyes in the original LA classification, 66.7% of Group D and 10.5% of Group E eyes which underwent globe preservation therapy with either systemic or intra-arterial chemotherapy avoided enucleation or radiotherapy (p<0.0001) (median follow-up of 33.0 months); 8.5% of Group D enucleated globes had high-risk histopathologic features while 26.3% of Group E globes had high risk features (p=0.0065). When Group D eyes with tumors meeting the size criteria were reclassified to Group E, 65.7-74.4% of Group D eyes and 16.1-36.7% of Group E eyes avoided enucleation or radiotherapy. Applying the tumor size criteria, 0-10.9% of Group D eyes had high-risk features.

Conclusions : Our retrospective analysis suggests that a large tumor size criteria for Group E retinoblastoma does not have significant clinical basis, given that the LA classification system provided the greatest separation in globe salvage rates between Group D and E eyes. The LA classification system also showed a statistically significant difference in the rates of high-risk features between Group D and E eyes. To avoid discrepancies in the literature, we recommend that centers use one uniform system for classifying advanced intraocular retinoblastoma.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

 

1. Treatment summary of eyes with advanced retinoblastoma (LA classification system and 10-18 mm tumor size criteria)

1. Treatment summary of eyes with advanced retinoblastoma (LA classification system and 10-18 mm tumor size criteria)

 

2. Summary of globe salvage rates for the LA classification system and the 10-18 mm tumor size criteria

2. Summary of globe salvage rates for the LA classification system and the 10-18 mm tumor size criteria

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