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Abstract：
Background: This study compares the outcomes of managing retinoblastoma between patients with unilateral and bilateral presentations. Methods: The study, conducted at the King Hussein Cancer Center in Amman, Jordan, retrospectively analyzed cases of retinoblastoma treated between March 2003 and December 2019. Evaluation criteria included clinical features, disease stage, treatment methods, and overall management outcomes. Results: The study comprised 697 eyes from 478 patients with retinoblastoma, with 52% being males. Bilateral disease was observed in 70% of patients, and a family history of retinoblastoma was more prevalent in cases with bilateral disease (20%) compared to those with unilateral disease (4%). Unilateral cases had a median age at diagnosis of 28 months, whereas bilateral cases were diagnosed at a median age of 6 months. Extra-ocular retinoblastoma was detected in 1% of eyes. According to the International Intraocular Retinoblastoma Classification (IIRC), 88% of unilateral cases presented with advanced disease (IIRC group D/E), compared to 46% in bilateral cases. Primary enucleation was performed in 29% of unilateral cases and 16% of bilateral cases (p-value 0.0007). Eye salvage rates were 31% in unilateral cases and 68% in bilateral cases (p-value < 0.0001). At 120 months of follow-up, 5% of patients died from secondary neoplasms or metastases, 81% were alive, and 14% were lost to follow-up. There was no significant difference in metastasis, secondary neoplasms, or mortality between patients with unilateral and bilateral retinoblastoma. Conclusions: This study highlights the nuanced differences in clinical characteristics and outcomes between unilateral and bilateral retinoblastoma, emphasizing the necessity of customized management and early detection strategies. It demonstrates that while bilateral retinoblastoma benefits from earlier detection and has a higher rate of eye salvage, there is no significant difference in metastasis or mortality rates when compared to unilateral cases. The critical roles of primary enucleation in advanced cases, along with effective communication and patient education, are also underscored to improve treatment adherence. Overall, these findings point to the importance of tailored approaches in optimizing outcomes for the diverse patient population affected by retinoblastoma.
摘要：
背景：这项研究比较了单侧和双侧表现的患者治疗视网膜母细胞瘤的结果。方法:研究,在安曼的侯赛因国王癌症中心进行，乔丹,回顾性分析2003年3月至2019年12月期间治疗的视网膜母细胞瘤病例.评价标准包括临床特征，疾病阶段,治疗方法,和整体管理成果。结果：该研究包括478例视网膜母细胞瘤患者的697只眼，52%是男性。在70％的患者中观察到双侧疾病，与患有单侧疾病的患者(4%)相比,患有双侧疾病的患者(20%)的视网膜母细胞瘤家族史更为普遍。单侧病例在诊断时的中位年龄为28个月，而双侧病例的诊断中位年龄为6个月。在1％的眼睛中检测到眼外视网膜母细胞瘤。根据国际眼内视网膜母细胞瘤分类（IIRC），88%的单侧病例表现为晚期疾病（IIRC组D/E），相比之下，双侧病例为46%。29％的单侧病例和16％的双侧病例进行了一次摘除（p值0.0007）。单侧病例的眼部抢救率为31%，双侧病例为68%(p值<0.0001)。在120个月的随访中，5%的患者死于继发性肿瘤或转移，81%还活着,14％的患者失去随访。在转移方面无显著差异,继发性肿瘤,或单侧和双侧视网膜母细胞瘤患者的死亡率。结论：本研究强调了单侧和双侧视网膜母细胞瘤在临床特征和结局方面的细微差别。强调定制管理和早期检测策略的必要性。它表明，虽然双侧视网膜母细胞瘤受益于早期检测，并且具有更高的眼部抢救率，与单侧病例相比，转移率或死亡率没有显着差异。原发性摘除术在晚期病例中的关键作用，以及有效的沟通和病人教育，还强调了提高治疗依从性。总的来说,这些发现表明,量身定制的方法对于优化受视网膜母细胞瘤影响的不同患者群体的结局具有重要意义.
