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

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: Ad...

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Detalles Bibliográficos
Autores: Tomar AS, Finger PT, Gallie B, Kivelä TT, Mallipatna A, Zhang C, Zhao J, Wilson MW, Brennan RC, Burges M, Kim J, Berry JL, Jubran R, Khetan V, Ganesan S, Yarovoy A, Yarovaya V, Kotova E, Volodin D, Yousef YA, Nummi K, Ushakova TL, Yugay OV, Polyakov VG, Ramirez-Ortiz MA, Esparza-Aguiar E, Chantada G, Schaiquevich P, Fandino A, Yam JC, Lau WW, Lam CP, Sharwood P, Moorthy S, Long QB, Essuman VA, Renner LA, Semenova E, Català-Mora J, Correa-Llano G, Carreras E
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p21447
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=21447
Access Level:acceso abierto
Palabra clave:Advanced
AJCC
Chemotherapy
Enucleation
International
Metastasis
Multicenter
Registry
Retinoblastoma
Staging
Descripción
Sumario: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. (C) 2022 by the American Academy of Ophthalmology.