Clinical Predictors at Diagnosis of Low-Risk Histopathology in Unilateral Advanced Retinoblastoma

Clinical Predictors at Diagnosis of Low-Risk Histopathology in Unilateral Advanced Retinoblastoma
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DOI:
10.1016/j.ophtha.2019.04.003
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发表时间:
2019-09-01
期刊:
影响因子:
13.7
通讯作者:
Soliman, Sameh E.
Soliman, Sameh E.
中科院分区:
医学1区
文献类型:
--
作者:
Kletke, Stephanie N.;Feng, Zhao Xun;Soliman, Sameh E.

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目的:与初次摘除术相比,尝试对单侧(cT2b/D 组)视网膜母细胞瘤进行眼部抢救可能存在肿瘤扩散的风险。识别预测低组织病理学风险的临床特征支持安全试验挽救。设计:回顾性、非比较性单机构观察病例系列。参与者:2008 年 1 月至 2018 年 2 月在加拿大多伦多病童医院接受初次摘除术的单侧 cT2b/D 组视网膜母细胞瘤儿童。方法:数据包括临床特征(眼压、视神经遮蔽、黄斑受累、主要结果指标:主要结果是低风险(LR;pT1/pT2)与高风险(HR;pT3/pT4)组织病理学特征以及临床病理学相关性。次要结果是阳性预测值(某些临床特征预测 LR 组织病理学特征的概率)和阴性预测值(缺乏这些临床特征将预测 HR 组织病理学特征的概率)。 结果:38 只眼睛符合条件,显示玻璃体种植和正常眼压。诊断到摘除间隔的中位时间为 4 天(范围,0-14 天)。组织病理学分析诊断出 4 只 (10.5%) HR 眼和 34 只 (89.5%) LR 眼。高危眼表现出大量脉络膜侵犯(4/38)或经巩膜、眼外和层后视神经侵犯(1/38)。临床结果包括黄斑受累 (31/38)、视神经完全遮蔽 (27/38) 和 RD (28/38)。具有 HR 组织病理学特征的眼睛比例为:黄斑受累为 13%(4/31;95% CI,1 %-25%),视神经完全遮蔽为 15%(4/27;95% CI,1 %-28%),RD 为 14%(4/28;95% CI,1 %-27%)。 LR 组织病理学特征的可预测性为 100%,黄斑保留(7/7;95% CI,47%-100%)、视神经可见度(10/10;95% CI,63%-100%)和 RD 小于 1 象限(10/10;95% CI,63%-100%)。 1 名儿童缺乏全部 3 种临床 LR 预测特征和 HR 组织病理学特征 (pT3a),发生转移并导致患者死亡;其他儿童均存活且状况良好(平均随访时间为 65 个月)。 结论:存在黄斑保留、视神经可见度、RD 小于 1 象限或其组合可预测初次摘除时的 LR 组织病理学特征,表明安全的抢救试验。没有临床体征预测 HR 组织病理学特征。 (C) 2019 年美国眼科学会
Purpose: Attempted eye salvage for unilateral (cT2b/group D) retinoblastoma may risk tumor spread compared with primary enucleation. Identification of clinical features predictive of low histopathologic risk support safe trial salvage.Design: Retrospective, noncomparative single-institutional observational case series.Participants: Children with unilateral cT2b/group D retinoblastoma managed with primary enucleation at the Hospital for Sick Children, Toronto, Canada, January 2008 through February 2018.Methods: Data included clinical features (intraocular pressure, optic nerve obscuration, macular involvement, tumor seeding, and serous retinal detachment [RD] >1 quadrant), timing to enucleation, histopathologic features, and follow-up.Main Outcome Measures: Primary outcome was low-risk (LR; pT1/pT2) versus high-risk (HR; pT3/pT4) histopathologic features with clinicopathologic correlations. Secondary outcomes were positive predictive (probability that certain clinical features would predict LR histopathologic features) and negative predictive values (probability that absence of these clinical features would predict HR histopathologic features).Results: Thirty-eight eyes were eligible and showed vitreous seeding and normal intraocular pressure. The median diagnosis to enucleation interval was 4 days (range, 0-14 days). Histopathologic analysis diagnosed 4 (10.5%) HR and 34 (89.5%) LR eyes. High-risk eyes demonstrated massive choroidal invasion (4/38) or trans-scleral, extraocular, and postlaminar optic nerve invasion (1/38). Clinical findings included macular involvement (31/38), complete optic nerve obscuration (27/38), and RD (28/38). The proportion of eyes with HR histopathologic features was 13% (4/31; 95% confidence interval [CI], 1 %-25%) with macular involvement, 15% (4/27; 95% CI, 1 %-28%) with complete optic nerve obscuration, and 14% (4/28; 95% CI, 1 %-27%) with RD. The predictability of LR histopathologic features was 100% with macular sparing (7/7; 95% CI, 47%-100%), optic nerve visibility (10/10; 95% CI, 63%-100%), and less than 1 quadrant of RD (10/10; 95% CI, 63%-100%). In 1 child lacking all 3 clinical LR predictive features with HR histopathologic features (pT3a), metastases developed and the patient died; other children are alive and well (mean follow-up, 65 months).Conclusions: Presence of macular sparing, optic nerve visibility, less than 1 quadrant of RD, or a combination thereof predicted LR histopathologic features at primary enucleation, suggesting safe trial eye salvage. No clinical sign predicted HR histopathologic features. (C) 2019 by the American Academy of Ophthalmology