Parrilla-Vallejo M, Perea-Pérez R, Relimpio-López I, Montero-de-Espinosa I, Rodríguez-de-la-Rúa E, Terrón-León J A, Díaz-Granda M J, Coca-Gutiérrez L, Ponte-Zuñiga B, Espejo-Arjona F
Oftalmología, Hospital Universitario Virgen Macarena, Sevilla, España.
Oftalmología, Hospital Universitario Virgen Macarena, Sevilla, España.
Arch Soc Esp Oftalmol (Engl Ed). 2018 Sep;93(9):423-430. doi: 10.1016/j.oftal.2018.04.010. Epub 2018 Jun 25.
Retinoblastoma is the most frequent malignant intraocular tumour in childhood, and both its cure and the sequelae arising from it, mainly depend on an early diagnosis. There is currently no consensus on its diagnostic and therapeutic management.
A descriptive, retrospective, and non-randomised study was conducted on a series of cases (39 patients -58 eyes), treated during the period 2006-2013, in the Regional Reference Centre for Tumours of the National Health Service Quality Agency.
The most frequent presentation sign is leukocoria (71.8%), followed by strabismus (17.9%). All cases of bilateral tumour had a germline mutation of the RB1 gene, and 20% had a family history. Stage E was observed in 55% of the patients, and 90% required chemotherapy treatment. The eye was maintained in 57% of those who had mild stages, compared to 43% who maintained it in advanced stages.
This analysis included 58 eyes. There are no previous studies in our community and there are few series so numerous throughout the country. Based on non-standardised treatment, the most appropriate is chosen according to the characteristics of the tumour. The multidisciplinary management, formed by ophthalmology, paediatric oncology, radiotherapy, and radiophysical oncology, is fundamental for the selection of the most appropriate treatment. Chemo-reduction, along with consolidation treatments, offers encouraging results in the control of these tumours, especially in those of less severity. Enucleation continues to be the method of choice in the most advanced staging with vitreous involvement, with the importance of early diagnosis being highlighted.
视网膜母细胞瘤是儿童期最常见的眼内恶性肿瘤，其治愈情况及由此产生的后遗症主要取决于早期诊断。目前对于其诊断和治疗管理尚无共识。
对2006年至2013年期间在国家卫生服务质量机构区域肿瘤参考中心接受治疗的一系列病例（39例患者 - 58只眼）进行了描述性、回顾性和非随机研究。
最常见的表现体征是白瞳症（71.8%），其次是斜视（17.9%）。所有双侧肿瘤病例均有RB1基因的种系突变，20%有家族史。55%的患者观察到E期，90%需要化疗。轻度分期患者中57%的患眼得以保留，而晚期患者中这一比例为43%。
本分析纳入了58只眼。我们社区此前没有相关研究，全国范围内如此大量病例的系列研究也很少。基于非标准化治疗，根据肿瘤特征选择最合适的治疗方法。由眼科、儿科肿瘤学、放射治疗和放射物理肿瘤学组成的多学科管理对于选择最合适的治疗至关重要。化疗减瘤以及巩固治疗在控制这些肿瘤方面取得了令人鼓舞的结果，尤其是对于病情较轻的肿瘤。在玻璃体受累的最晚期分期中，眼球摘除术仍然是首选方法，同时强调了早期诊断的重要性。
