Retinoblastoma with and without Extraocular Tumor Extension

IF 3.2 Q1 OPHTHALMOLOGY

Ophthalmology science Pub Date : 2024-10-30 DOI:10.1016/j.xops.2024.100637

Swathi Kaliki MD , Vijitha S. Vempuluru MD , Ido Didi Fabian MD

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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.

Financial Disclosure(s)

The authors have no proprietary or commercial interest in any materials discussed in this article.

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伴有和不伴有眼外肿瘤扩展的视网膜母细胞瘤：3435例患者的全球比较研究

目的：探讨伴有眼外肿瘤扩展（RB- eoe）的儿童视网膜母细胞瘤（RB）的治疗及转归，并与不伴有眼外肿瘤扩展（RB-w/o-EOE）的儿童视网膜母细胞瘤（RB）进行比较。设计：2017 - 2020年多中心洲际合作前瞻性研究。RB-EOE病例包括未接受治疗的眼眶肿瘤明显扩展的患者。在显微镜下发现眼眶扩张的病例被排除在研究之外。参与者：共319名RB-EOE患儿和3116名RB-w/o-EOE患儿。干预：化疗、去核、拔管、放疗。主要观察指标：全身转移和死亡。结果：在本研究纳入的3435例RB患者中，309例（9%）来自低收入国家（LIC）， 1448例（42%）来自中低收入国家，1012例（29%）来自中高收入国家，666例（19%）来自高收入国家。RB-EOE的百分比与国民收入水平呈负相关，低收入国家96例（31%），中低收入国家197例（6%），中高收入国家20例（2%），高收入国家6例（1%）（P = 0.0001）。与RB-w/o-EOE相比，RB-EOE的结果具有统计学意义：系统性转移(分别为32% vs. 4%；P = 0.0001)和转移相关死亡(分别为63% vs. 6%；P = 0.0001)。多模式治疗是最常见的治疗形式(n = 177；54%)为RB-EOE，大多数病例接受静脉化疗和去核联合治疗(n = 97；30%)。术后辅助外束放疗（EBRT）（去核/眼眶摘除）仅68例（21%）。Kaplan-Meier分析显示，RB-EOE患者1年的系统性转移和转移相关死亡分别为28%和57%，2年为29%和60%，3年为29%和61%。Cox回归分析显示，bb0 ~ 4岁患者RB-EOE的死亡风险高于P < 0.001，单峰（手术或静脉化疗）和双峰（手术和静脉化疗）治疗的死亡风险高于三峰（手术、静脉化疗和EBRT）治疗(风险比，2.023；P = 0.004，风险比为1.819；P = 0.027)。结论：伴有眼外肿瘤扩展的视网膜母细胞瘤具有较高的转移和死亡风险。RB-EOE患者可能受益于三模式治疗（静脉化疗、手术和EBRT），而不是不包括EBRT的治疗方案。财务披露：作者在本文中讨论的任何材料中没有专有或商业利益。

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