Naseripour M, Nazari H, Bakhtiari P, Modarres-zadeh M, Vosough P, Ausari M
Eye Research Center, Iran University of Medical Sciences, Niayesh Avenue, Sattarkhan Street, Tehran, Iran.
Br J Ophthalmol. 2009 Jan;93(1):28-32. doi: 10.1136/bjo.2008.139410. Epub 2008 Oct 24.
BACKGROUND/AIMS: Retinoblastoma is a highly malignant eye tumour in children with different survival rates across the world. The aims of this study are to determine the globe and patient survival in children with retinoblastoma in a major referral centre in Iran.
156 eyes of 105 consecutive patients with retinoblastoma were enrolled from 2001 to 2007. All demographic data, family history, presenting symptoms, duration of symptoms, ocular findings and treatment modalities that were used for the patients were collected. For patient survival, event was defined as death and for globe survival as enucleation.
The mean age at diagnosis was 28.5 months (unilateral 27.4 months; bilateral 30 months). Five patients had a positive family history. Fifty-two per cent of the cases were unilateral, and 48% were bilateral. The most common presenting sign was leucocoria (64.8%) followed by strabismus (28.2%). Enucleation was done primarily for 75.9% of unilateral cases and 34.3% of bilateral cases. Secondary enucleation was necessary in 5.6% and 7.8% of unilateral and bilaterally involved eyes respectively. Sixty-nine (44.2%) of 156 eyes were salvaged by different globe preserving modalities (unilateral 18.5%; bilateral 57.9%). The Kaplan-Meier survival estimate for globe preservation according to International Classification of Retinoblastoma (ICRB) was 100% for group A eyes, 93.5% for group B, 86.7% for group C, 57.1% for group D and 0% for group E eyes. Kaplan-Meier estimates for patients survival were 100% at 1 year, 94.8% at 3 years and 83.1% at 5 years.
Progress in methods of treatment, early detection of the disease and prompt referral to specialised centres have led to improved outcomes for patients with retinoblastoma in terms of globe and patients' survival rates even in developing countries.
背景/目的：视网膜母细胞瘤是一种儿童眼部高度恶性肿瘤，全球生存率各异。本研究旨在确定伊朗一家主要转诊中心视网膜母细胞瘤患儿的眼球和患者生存率。
2001年至2007年纳入105例连续的视网膜母细胞瘤患者的156只眼。收集所有患者的人口统计学数据、家族史、就诊症状、症状持续时间、眼部检查结果及治疗方式。患者生存方面，事件定义为死亡；眼球生存方面，事件定义为眼球摘除。
诊断时的平均年龄为28.5个月（单眼27.4个月；双眼30个月）。5例患者有阳性家族史。52%的病例为单眼，48%为双眼。最常见的就诊体征是白瞳症（64.8%），其次是斜视（28.2%）。75.9%的单眼病例和34.3%的双眼病例首先进行了眼球摘除。单眼和双眼受累眼分别有5.6%和7.8%需要进行二期眼球摘除。156只眼中有69只（44.2%）通过不同的保眼方式得以保留（单眼18.5%；双眼57.9%）。根据国际视网膜母细胞瘤分类（ICRB），A组眼球保眼的Kaplan-Meier生存估计为100%，B组为93.5%，C组为86.7%，D组为57.1%，E组为0%。患者生存的Kaplan-Meier估计为1年时100%，3年时94.8%，5年时83.1%。
治疗方法的进步、疾病的早期发现以及及时转诊至专科中心，即使在发展中国家，也使视网膜母细胞瘤患者在眼球和患者生存率方面的预后得到改善。
