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对482例连续性单侧视网膜母细胞瘤患者新肿瘤形成情况的条件分析。

Conditional analysis on new tumor formation with solitary unilateral retinoblastoma in 482 consecutive patients.

作者信息

Shields Carol L, Dockery Philip W, Ruben Megan, Sunday Madalyne A, Calotti Martin, Yaghy Antonio

机构信息

Ocular Oncology Services, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.

出版信息

Saudi J Ophthalmol. 2022 Jun 13;35(4):279-285. doi: 10.4103/sjopt.sjopt_146_21. eCollection 2021 Oct-Dec.

Abstract

PURPOSE

The objective of the study was to understand dynamic risk (conditional analysis based on patient age) for new tumor development in patients with solitary unilateral retinoblastoma.

METHODS

This was a retrospective analysis.

RESULTS

Of 482 patients with solitary unilateral retinoblastoma, 55 new tumors developed in 20 patients (4%). Comparison (new tumor vs. no new tumor development) revealed those with new tumor demonstrated younger mean age at presentation (10 vs. 36 months, < 0.001), greater likelihood of family history of retinoblastoma (35% vs. 3%, < 0.001), and greater probability of primary tumor location in the macula (50% vs. 15%, = 0.003). Conditional risk for new tumors (at age 6, 9, 12, 18, and 24 months) declined for those who presented at 0-3 months old (25%, 15%, 15%, 8%, and 0%), >3-6 months old (17%, 14%, 6%, 6%, and 0%), >6-9 months old (not applicable [na], 6%, 6%, 0%, and 0%), and >9-12 months (na, na, 3%, 3%, and 0%). Younger patients showed greater development of bilateral tumors ( < 0.001). Of patients with new tumors, those that occurred within 1 year from presentation were located in the preequatorial region in 46%, whereas those that occurred more than 1 year from presentation were preequatorial in 78%. Patients ≤24 months at initial presentation demonstrated all new tumors by 24 months of age. Older patients (>24 months at presentation) showed new tumors up to 56 months of age.

CONCLUSION

Children (≤24 months) with solitary unilateral retinoblastoma showed decreasing risk for new tumors up to 24 months of life. Later onset of new tumor was more likely located in preequatorial region.

摘要

目的

本研究的目的是了解单侧孤立性视网膜母细胞瘤患者发生新肿瘤的动态风险(基于患者年龄的条件分析)。

方法

这是一项回顾性分析。

结果

在482例单侧孤立性视网膜母细胞瘤患者中,20例(4%)出现了55个新肿瘤。比较(有新肿瘤与无新肿瘤发生)发现,有新肿瘤的患者就诊时平均年龄更小(10个月对36个月,<0.001),视网膜母细胞瘤家族史的可能性更大(35%对3%,<0.001),原发肿瘤位于黄斑区的概率更高(50%对15%,=0.003)。0至3个月就诊的患者发生新肿瘤的条件风险(在6、9、12、18和24个月时)下降(25%、15%、15%、8%和0%),>3至6个月就诊的患者(17%、14%、6%、6%和0%),>6至9个月就诊的患者(不适用[na],6%、6%、0%和0%),以及>9至12个月就诊的患者(na、na、3%、3%和0%)。年龄较小的患者双侧肿瘤发生率更高(<0.001)。在有新肿瘤的患者中,就诊后1年内发生的新肿瘤46%位于赤道前区域,而就诊后1年以上发生的新肿瘤78%位于赤道前区域。初次就诊时年龄≤24个月的患者在24个月龄时出现了所有新肿瘤。年龄较大的患者(就诊时>24个月)在56个月龄时出现新肿瘤。

结论

单侧孤立性视网膜母细胞瘤患儿(≤24个月)在24个月龄前发生新肿瘤的风险逐渐降低。新肿瘤较晚发生更可能位于赤道前区域。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6cfd/9266472/b3ca736291a0/SJO-35-279-g001.jpg

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Likelihood of Germline Mutation With Solitary Unilateral Retinoblastoma Based on Patient Age at Presentation: Analysis of 482 Consecutive Patients.
J Pediatr Ophthalmol Strabismus. 2021 Nov-Dec;58(6):355-364. doi: 10.3928/01913913-20210414-02. Epub 2021 Jun 28.
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