20.500.12556/DiRROS-28105 Retinoblastoma with and without extraocular tumor extension a global comparative study of 3435 patients Purpose To study the treatment and outcomes of children with retinoblastoma (RB) with extraocular tumor extension (RB-EOE) and compare them with RB without extraocular tumor extension (RB-w/o-EOE). Design Multicenter intercontinental collaborative prospective study from 2017 to 2020. RB-EOE cases included those with overt orbital tumor extension in treatment-naive patients. Cases with microscopic orbital extension detected postenucleation were excluded from the study. Participants A total of 319 children with RB-EOE and 3116 children with RB-w/o-EOE. Intervention Chemotherapy, enucleation, exenteration, radiotherapy. Main Outcome Measures Systemic metastasis and death. Results Of the 3435 RB patients included in this study, 309 (9%) were from low-income countries (LIC), 1448 (42%) from lower-middle income, 1012 (29%) from upper-middle income, and 666 (19%) patients from high-income countries. There was an inverse relationship between the percentage of RB-EOE and national income level, with 96 (31%) patients from LIC, 197 (6%) lower-middle income, 20 (2%) upper-middle income, and 6 (1%) patients from high-income countries (P = 0.0001). The outcomes were statistically significant for RB-EOE compared with RB-w/o-EOE: systemic metastasis (32% vs. 4% respectively; P = 0.0001) and metastasis-related death (63% vs. 6% respectively; P = 0.0001). Multimodal treatment was the most common form of treatment (n = 177; 54%) for RB-EOE, with most cases undergoing a combination of intravenous chemotherapy and enucleation (n = 97; 30%). Adjuvant external beam radiotherapy (EBRT) after surgery (enucleation/orbital exenteration) was given in only 68 (21%) cases. Kaplan–Meier analysis for systemic metastasis and metastasis-related death in RB-EOE was 28% and 57% at 1 year, 29% and 60% at 2 years, and 29% and 61% at 3 years, respectively. Cox regression analysis revealed that the risk of death from RB-EOE was greater in patients aged >4 years than <2 years (hazard ratio, 2.912; P < 0.001) and for unimodal (surgery or intravenous chemotherapy) and bimodal (surgery and intravenous chemotherapy) treatment than trimodal treatment (surgery, intravenous chemotherapy, and EBRT) (hazard ratio, 2.023; P = 0.004 and hazard ratio, 1.819; P = 0.027, respectively). Conclusions Retinoblastoma with extraocular tumor extension is associated with a higher risk of metastasis and death. Patients with RB-EOE are likely to benefit from trimodal treatment (intravenous chemotherapy, surgery, and EBRT) rather than treatment protocols excluding EBRT. external beam radiotherapy extraocular extension multimodal treatment retinoblastoma tumor obsevanje z zunanjim žarkom zunajočesna ekstenzija multimodalno zdravljenje retinoblastom tumor true false true Angleški jezik Slovenski jezik Neznano 2026-03-10 11:02:40 2026-03-10 11:02:40 2026-03-11 03:59:15 0000-00-00 00:00:00 2025 0 0 Nasl. z nasl. zaslona; Opis vira z dne 27. 6. 2025; str. 1-8 issue 2, [article no.] 100637 Vol. 5 2025 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 617.7 2666-9145 10.1016/j.xops.2024.100637 240654851 RAZ_Kaliki_Swathi_2025.pdf RAZ_Kaliki_Swathi_2025.pdf 1 0E2454203BDEDAFC08B119F605F79664 3c26f9f30e0381b0f23bbe9966d3c8dd97befcbaf18df6f597683eb9cb59645c 78b57589-1c68-11f1-acf8-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=41535 https://doi.org/10.1016/j.xops.2024.100637 0 46d61f7e-1c68-11f1-acf8-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=41534 Univerzitetni klinični center Ljubljana 0 0 0