Lin Phoebe, O'Brien Joan M
Department of Ophthalmology, University of California, San Francisco, San Francisco, CA 94143, USA.
Am J Ophthalmol. 2009 Aug;148(2):192-8. doi: 10.1016/j.ajo.2009.04.004. Epub 2009 May 24.
To provide an overview of the current clinical management of retinoblastoma by discussing the trends in the categorization, treatment, and recent advances in molecular diagnostics as well as therapy for retinoblastoma.
Literature review and commentary.
Selected articles from the medical literature and the authors' clinical and research experience were reviewed critically.
Retinoblastoma has evolved from a deadly childhood cancer to a largely curable cancer within the past 40 years. Current treatment strategies aim to salvage the eye and provide the best visual outcome possible. Using the international classification system to stratify intraocular retinoblastoma into treatment groups, the multicenter Children's Oncology Group treatment protocols use 2- to 3-drug chemoreduction with focal consolidative therapy for most categories of disease. Furthermore, collaborative efforts are being directed toward a better understanding of genotype-phenotype relationships in retinoblastoma that will be useful in the multidisciplinary management of this disease. Molecular targeting therapy is emerging as a potential strategy to individualize therapy. Finally, improvements in local drug delivery methods and vehicles are providing solutions for the problem of systemic toxicity from existing chemotherapy regimens.
The management of retinoblastoma has become a prototype for other ophthalmic diseases and systemic cancers in which genetic information and molecular targets are being used to design more elegant treatment strategies.
通过讨论视网膜母细胞瘤在分类、治疗方面的趋势以及分子诊断和治疗的最新进展，对视网膜母细胞瘤当前的临床管理进行概述。
文献综述与评论。
对从医学文献中选取的文章以及作者的临床和研究经验进行严格审查。
在过去40年里，视网膜母细胞瘤已从一种致命的儿童癌症演变为一种基本上可治愈的癌症。当前的治疗策略旨在挽救眼球并尽可能提供最佳视觉效果。利用国际分类系统将眼内视网膜母细胞瘤分层到不同治疗组，多中心儿童肿瘤协作组的治疗方案对大多数疾病类型采用2至3种药物的化学减积疗法并结合局部巩固治疗。此外，正在开展合作努力以更好地理解视网膜母细胞瘤的基因型 - 表型关系，这将有助于对该疾病进行多学科管理。分子靶向治疗正成为一种使治疗个体化的潜在策略。最后，局部给药方法和载体的改进正在为现有化疗方案的全身毒性问题提供解决方案。
视网膜母细胞瘤的管理已成为其他眼科疾病和全身性癌症的典范，在这些疾病中，遗传信息和分子靶点正被用于设计更优化的治疗策略。
