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单侧视网膜母细胞瘤;248 例患者的自然病史和基于年龄的方案。

Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients.

机构信息

Egyptian National Cancer Institute, Cairo, Egypt.

Children's Cancer Hospital Egypt, Cairo, 57357, Egypt.

出版信息

Eye (Lond). 2021 Sep;35(9):2564-2572. doi: 10.1038/s41433-020-01275-2. Epub 2020 Nov 13.

DOI:10.1038/s41433-020-01275-2
PMID:33188294
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8377076/
Abstract

OBJECTIVES

We aimed to study the clinical state and prognosis of patients with unilateral retinoblastoma who were being treated at a paediatric comprehensive cancer centre in a limited-resource country, to assess the different phases of treatment and the success of different, more complex real-life models.

SUBJECTS

In this retrospective study, we created a snapshot of our retinoblastoma database for the period between 2007 and 2015. Patients whose data were included in the study were followed up until 2016. Out of a total of 744 screened patients, we included data of 248 patients who had been diagnosed with unilateral retinoblastoma.

RESULTS

As classified as per the International Retinoblastoma Classification, 1 patient presented with group A, 21 with group B, 39 with group C, 104 with group D and 83 with group E retinoblastoma. Chemotherapy was the initial line of treatment in 115 patients and enucleation in 133 others. Later, 141 patients (56.9%) required further management. Patients had a mean ocular survival time of 20.8 months. Nine patients developed extraocular disease at a later stage of management: five after upfront enucleation and four after neoadjuvant chemotherapy. Mean overall survival time stood at 90.2 months. Four and three deaths were recorded in groups D and E, respectively. A single patient died in the initial chemotherapy arm, while six passed away in the initial enucleation arm.

CONCLUSION

Our study highlights the importance of initial chemotherapy and close follow-up after enucleation of classes D and E affected eyes even in absence of germline mutations.

摘要

目的

我们旨在研究在资源有限的国家的儿科综合癌症中心接受治疗的单侧视网膜母细胞瘤患者的临床状况和预后,评估不同治疗阶段和不同、更复杂的真实模型的成功率。

对象

在这项回顾性研究中,我们为 2007 年至 2015 年期间的视网膜母细胞瘤数据库创建了一个快照。纳入研究的患者一直随访到 2016 年。在总共筛选出的 744 名患者中,我们纳入了 248 名单侧视网膜母细胞瘤患者的数据。

结果

根据国际视网膜母细胞瘤分类,1 名患者为 A 组,21 名患者为 B 组,39 名患者为 C 组,104 名患者为 D 组,83 名患者为 E 组视网膜母细胞瘤。115 名患者最初接受化疗,133 名患者接受眼球摘除术。随后,141 名患者(56.9%)需要进一步治疗。患者的平均眼部生存时间为 20.8 个月。9 名患者在管理的后期发生眼外疾病:5 名在初次眼球摘除后,4 名在新辅助化疗后。总的平均生存时间为 90.2 个月。D 组和 E 组分别有 4 例和 3 例死亡。1 名患者在初始化疗组死亡,6 名患者在初始眼球摘除组死亡。

结论

我们的研究强调了即使没有种系突变,在 D 类和 E 类受影响眼睛进行初始化疗和眼球摘除后的密切随访的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/10e396485e57/41433_2020_1275_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/a472775c14fb/41433_2020_1275_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/01a88d6c6e02/41433_2020_1275_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/10e396485e57/41433_2020_1275_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/a472775c14fb/41433_2020_1275_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/01a88d6c6e02/41433_2020_1275_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d711/8377076/10e396485e57/41433_2020_1275_Fig3_HTML.jpg

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J Clin Oncol. 2022 Nov 20;40(33):3839-3847. doi: 10.1200/JCO.21.02337. Epub 2022 Jul 12.
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Global Retinoblastoma Presentation and Analysis by National Income Level.按国民收入水平对全球视网膜母细胞瘤的呈现与分析
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Study of Unilateral Retinoblastoma With and Without Histopathologic High-Risk Features and the Role of Adjuvant Chemotherapy: A Children's Oncology Group Study.
Management of Intraocular Retinoblastoma: ICMR Consensus Guidelines.
眼内视网膜母细胞瘤的管理:ICMR 共识指南。
Indian J Pediatr. 2024 Nov;91(11):1166-1176. doi: 10.1007/s12098-024-05095-0. Epub 2024 Apr 13.
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