Metastatic Death Based on Presenting Features and Treatment for Advanced Intraocular Retinoblastoma: A Multicenter Registry-Based Study.

Abstract

PURPOSE:

To evaluate presenting features, tumor size, and treatment methods for risk of metastatic death due to advanced intraocular retinoblastoma (RB).

DESIGN:

International, multicenter, registry-based retrospective case series.

PARTICIPANTS:

A total of 1841 patients with advanced RB.

METHODS:

Advanced RB was defined by 8th edition American Joint Committee on Cancer (AJCC) categories cT2 and cT3 and new AJCC-Ophthalmic Oncology Task Force (OOTF) Size Groups (1: < 50% of globe volume, 2: > 50% but < 2/3, 3: > 2/3, and 4: diffuse infiltrating RB). Treatments were primary enucleation, systemic chemotherapy with secondary enucleation, and systemic chemotherapy with eye salvage.

MAIN OUTCOME MEASURES:

Metastatic death.

RESULTS:

The 5-year Kaplan-Meier cumulative survival estimates by patient-level AJCC clinical subcategories were 98% for cT2a, 96% for cT2b, 88% for cT3a, 95% for cT3b, 92% for cT3c, 84% for cT3d, and 75% for cT3e RB. Survival estimates by treatment modality were 96% for primary enucleation, 89% for systemic chemotherapy and secondary enucleation, and 90% for systemic chemotherapy with eye salvage. Risk of metastatic mortality increased with increasing cT subcategory (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of metastatic mortality in categories cT3c (glaucoma, hazard ratio [HR], 4.9; P = 0.011), cT3d (intraocular hemorrhage, HR, 14.0; P < 0.001), and cT3e (orbital cellulitis, HR, 19.6; P < 0.001) than in category cT2a and with systemic chemotherapy with secondary enucleation (HR, 3.3; P < 0.001) and eye salvage (HR, 4.9; P < 0.001) than with primary enucleation. The 5-year Kaplan-Meier cumulative survival estimates by AJCC-OOTF Size Groups 1 to 4 were 99%, 96%, 94%, and 83%, respectively. Mortality from metastatic RB increased with increasing Size Group (P < 0.001). Cox proportional hazards regression analysis revealed that patients with Size Group 3 (HR, 10.0; P = 0.002) and 4 (HR, 41.1; P < 0.001) had a greater risk of metastatic mortality than Size Group 1.

CONCLUSIONS:

The AJCC-RB cT2 and cT3 subcategories and size-based AJCC-OOTF Groups 3 (> 2/3 globe volume) and 4 (diffuse infiltrating RB) provided a robust stratification of clinical risk for metastatic death in advanced intraocular RB. Primary enucleation offered the highest survival rates for patients with advanced intraocular RB.

• Authors: Ankit Singh Tomar, Paul T Finger, Brenda Gallie, Tero T Kivelä, Ashwin Mallipatna, Chengyue Zhang, Junyang Zhao, Matthew W Wilson, Rachel C Brennan, Michala Burges, Jonathan Kim, Jesse L Berry, Rima Jubran, Vikas Khetan, Suganeswari Ganesan, Andrey Yarovoy, Vera Yarovaya, Elena Kotova, Denis Volodin, Yacoub A Yousef, Kalle Nummi, Tatiana L Ushakova, Olga V Yugay, Vladimir G Polyakov, Marco A Ramirez-Ortiz, Elizabeth Esparza-Aguiar, Guillermo Chantada, Paula Schaiquevich, Adriana Fandino, Jason C Yam, Winnie W Lau, Carol P Lam, Phillipa Sharwood, Sonia Moorthy, Quah Boon Long, Vera Adobea Essuman, Lorna A Renner, Ekaterina Semenova, Jaume Català-Mora, Genoveva Correa-Llano, Elisa Carreras, American Joint Committee on Cancer Ophthalmic Oncology Task Force
• Journal: Ophthalmology (Ophthalmology) Vol. 129 Issue 8 Pg. 933-945 (08 2022) ISSN: 1549-4713 [Electronic] United States
• PMID: 35500608 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Topics

• Eye Enucleation
• Humans
• Infant
• Registries
• Retinal Neoplasms (drug therapy, pathology)
• Retinoblastoma (drug therapy, pathology)
• Retrospective Studies