Kim Hyun Ah, Yoon Young Hee, Kim June Gone, Lee Joo Yong
Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Ophthalmol. 2020 Jun;34(3):242-250. doi: 10.3341/kjo.2019.0097.
We aimed to study the clinical characteristics and long-term prognoses of retinoblastoma according to the age at diagnosis.
A retrospective chart review of non-screened patients newly diagnosed with retinoblastoma between January 2007 and February 2018.
Among the 20 patients analyzed, 11 were diagnosed at an age younger than 1 year (group 1) and nine at 1 year or older (group 2). The mean lag times until diagnosis were 1.0 ± 0.4 and 5.0 ± 2.1 months for groups 1 and 2, respectively ( = 0.056). The mean follow-up durations were 49.4 ± 12.7 and 58.3 ± 8.8 months, respectively ( = 0.412). Group 1 had a significantly higher proportion of bilateral retinoblastoma than did group 2 (72.7% vs. 11.1%, = 0.010). Four of five patients (80.0%) with germline mutations were diagnosed with retinoblastoma at age 3 months or younger. The eyes of patients in group 2 had significantly higher International Intraocular Retinoblastoma Classification stages than did those of patients in group 1 ( for trend = 0.010). The proportion of eyes with optic nerve invasion and those that had undergone enucleation were significantly higher in group 2 ( = 0.033 and 0.046, respectively). Survival did not differ according to the age at diagnosis.
Early onset retinoblastoma does not seem to indicate poor ocular or survival prognosis in Korean children with retinoblastoma.
我们旨在根据诊断时的年龄研究视网膜母细胞瘤的临床特征和长期预后。
对2007年1月至2018年2月新诊断为视网膜母细胞瘤的未筛查患者进行回顾性病历审查。
在分析的20例患者中，11例在1岁以下被诊断（第1组），9例在1岁或以上被诊断（第2组）。第1组和第2组诊断前的平均延迟时间分别为1.0±0.4个月和5.0±2.1个月（P = 0.056）。平均随访时间分别为49.4±12.7个月和58.3±8.8个月（P = 0.412）。第1组双侧视网膜母细胞瘤的比例显著高于第2组（72.7%对11.1%，P = 0.010）。5例（80.0%）种系突变患者中有4例在3个月或更小年龄被诊断为视网膜母细胞瘤。第2组患者眼睛的国际眼内视网膜母细胞瘤分类阶段显著高于第1组患者（趋势P = 0.010）。第2组视神经侵犯和已摘除眼球的比例显著更高（分别为P = 0.033和0.046）。生存情况根据诊断时的年龄没有差异。
在韩国视网膜母细胞瘤患儿中，早发性视网膜母细胞瘤似乎并不表明眼部或生存预后不良。
