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Multicenter Study
. 2022 Aug;129(8):923-932.
doi: 10.1016/j.ophtha.2022.04.006. Epub 2022 Apr 15.

High-risk Pathologic Features Based on Presenting Findings in Advanced Intraocular Retinoblastoma: A Multicenter, International Data-Sharing American Joint Committee on Cancer Study

Ankit Singh Tomar  1 , Paul T Finger  2 , Brenda Gallie  3 , Tero T Kivelä  4 , Ashwin Mallipatna  5 , Chengyue Zhang  6 , Junyang Zhao  6 , Matthew W Wilson  7 , Rachel C Brennan  7 , Michala Burges  7 , Jonathan Kim  8 , Jesse L Berry  8 , Rima Jubran  8 , Vikas Khetan  9 , Suganeswari Ganesan  9 , Andrey Yarovoy  10 , Vera Yarovaya  10 , Elena Kotova  10 , Denis Volodin  10 , Yacoub A Yousef  11 , Kalle Nummi  4 , Tatiana L Ushakova  12 , Olga V Yugay  13 , Vladimir G Polyakov  12 , Marco A Ramirez-Ortiz  14 , Elizabeth Esparza-Aguiar  14 , Guillermo Chantada  15 , Paula Schaiquevich  16 , Adriana Fandino  17 , Jason C Yam  18 , Winnie W Lau  18 , Carol P Lam  18 , Phillipa Sharwood  19 , Sonia Moorthy  20 , Quah Boon Long  20 , Vera Adobea Essuman  21 , Lorna A Renner  22 , Ekaterina Semenova  1 , Jaume Català-Mora  23 , Genoveva Correa-Llano  23 , Elisa Carreras  23 ; American Joint Committee on Cancer Ophthalmic Oncology Task Force
Affiliations
Multicenter Study

High-risk Pathologic Features Based on Presenting Findings in Advanced Intraocular Retinoblastoma: A Multicenter, International Data-Sharing American Joint Committee on Cancer Study

Ankit Singh Tomar et al. Ophthalmology. 2022 Aug.

Abstract

Purpose: To determine the value of clinical features for advanced intraocular retinoblastoma as defined by the eighth edition of the American Joint Committee on Cancer (AJCC) cT3 category and AJCC Ophthalmic Oncology Task Force (OOTF) Size Groups to predict the high-risk pathologic features.

Design: International, multicenter, registry-based retrospective case series.

Participants: Eighteen ophthalmic oncology centers from 13 countries over 6 continents shared evaluations of 942 eyes enucleated as primary treatment for AJCC cT3 and, for comparison, cT2 retinoblastoma.

Methods: International, multicenter, registry-based data were pooled from patients enrolled between 2001 and 2013. High-risk pathologic features were defined as AJCC categories pT3 and pT4. In addition, AJCC OOTF Size Groups were defined as follows: (1) less than half, (2) more than half but less than two thirds, (3) more than two thirds of globe volume involved, and (4) diffuse infiltrating retinoblastoma.

Main outcome measures: Statistical risk of high-risk pathologic features corresponding to AJCC cT3 subcategories and AJCC OOTF Size Groups.

Results: Of 942 retinoblastoma eyes treated by primary enucleation, 282 (30%) showed high-risk pathologic features. Both cT subcategories and AJCC OOTF Size Groups (P < 0.001 for both) were associated with high-risk pathologic features. On logistic regression analysis, cT3c (iris neovascularization with glaucoma), cT3d (intraocular hemorrhage), and cT3e (aseptic orbital cellulitis) were predictive factors for high-risk pathologic features when compared with cT2a with an odds ratio of 2.3 (P = 0.002), 2.5 (P = 0.002), and 3.3 (P = 0.019), respectively. Size Group 3 (more than two-thirds globe volume) and 4 (diffuse infiltrative retinoblastoma) were the best predictive factors with an odds ratio of 3.3 and 4.1 (P < 0.001 for both), respectively, for high-risk pathologic features when compared with Size Groups 1 (i.e., < 50% of globe volume).

Conclusions: The AJCC retinoblastoma staging clinical cT3c-e subcategories (glaucoma, intraocular hemorrhage, and aseptic orbital cellulitis, respectively) as well as the AJCC OOTF Size Groups 3 (tumor more than two thirds of globe volume) and 4 (diffuse infiltrative retinoblastoma) both allowed stratification of clinical risk factors that can be used to predict the presence of high-risk pathologic features and thus facilitate treatment decisions.

Keywords: AJCC; Multicenter; Pathology; Retinoblastoma; Staging.

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Conflict of interest statement

Disclosure(s):

All authors have completed and submitted the ICMJE disclosures form.

The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Supported by The Myrna and John Daniels Charitable Trust, the Paul T. Finger Fund, and The Eye Cancer Foundation, which provided monetary support to the Princess Margaret Cancer Centre’s Internet Technology Program, which in turn participated in the design, construction, and maintenance of this retinoblastoma registry; and The Eye Cancer Foundation (A.S.T.). The funding sources had no role in the design and conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; or decision to submit the manuscript for publication.

Figures

Figure 1.
Figure 1.
Consolidated Standards for Reporting Trials flow diagram showing all eyes with advanced retinoblastoma (RB) treated with primary enucleation. AJCC = American Joint Committee on Cancer; OOTF = Ophthalmic Oncology Task Force.
See this image and copyright information in PMC

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