| Title: | Retinoblastoma with and without extraocular tumor extension : a global comparative study of 3435 patients |
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| Authors: | ID Kaliki, Swathi (Author)
ID Vempuluru, Vijitha S. (Author)
ID Fabian, Ido Didi (Author)
ID Lavrič Groznik, Alenka (Research coworker)
ID Tekavčič Pompe, Manca (Research coworker), et al. |
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| Files: |  PDF - Presentation file, download (508,94 KB)
MD5: 0E2454203BDEDAFC08B119F605F79664
 URL - Source URL, visit https://doi.org/10.1016/j.xops.2024.100637
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| Language: | English |
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| Typology: | 1.01 - Original Scientific Article |
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| Organization: |  UKC LJ - Ljubljana University Medical Centre
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| Abstract: | 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. |
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| Keywords: | external beam radiotherapy, extraocular extension, multimodal treatment, retinoblastoma, tumor |
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| Publication status: | Published |
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| Publication version: | Version of Record |
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| Year of publishing: | 2025 |
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| Number of pages: | str. 1-8 |
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| Numbering: | Vol. 5, issue 2, [article no.] 100637 |
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| PID: | 20.500.12556/DiRROS-28105  |
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| UDC: | 617.7 |
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| ISSN on article: | 2666-9145 |
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| DOI: | 10.1016/j.xops.2024.100637 |
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| COBISS.SI-ID: | 240654851 |
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| Note: | Nasl. z nasl. zaslona;
Opis vira z dne 27. 6. 2025;
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| Publication date in DiRROS: | 10.03.2026 |
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| Views: | 75 |
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| Downloads: | 37 |
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