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

ANKIT SINGH TOMAR; PAUL T. FINGER; BRENDA GALLIE; TERO T. KIVELÄ; CHANTADA G; SCHAIQUEVICH P; JAUME CATALÀ-MORA; GENOVEVA CORREA-LLANO ; ELISA CARRERAS

NEURO-OPHTHALMOLOGY

TAYLOR & FRANCIS INC

Lugar: Londres; Año: 2022

0165-8107

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: 1841 patients with advanced RB.Methods: Patients were registered between 2001 and 2013. Advanced RB was defined by 8th edition AJCC categories cT2 and cT3 as well as new AJCC-Ophthalmic Oncology Task Force(OOTF) Size Groups (Group 1: 50% but 2/3, and Group 4: diffuse infiltrating retinoblastoma). Treatments were primary enucleation, systemic chemotherapy with secondary enucleation, and systemic chemotherapy with eye salvage.Main outcome measures: Metastatic death estimated by Kaplan-Meier analysis and Cox proportional hazards regression.Results: 5-year Kaplan-Meier cumulative survival estimates by patient-level AJCC clinical subcategories were 98% for cT2a, 96% cT2b, 88% cT3a, 95% cT3b, 92% cT3c, 84% cT3d, and 75% for cT3e RB. Survival estimates by treatment modality were 96% for primary enucleation, 89% systemic chemotherapy and secondary enucleation, and 90% 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