视网膜母细胞瘤的综合治疗策略

视网膜母细胞瘤（RB）是婴幼儿眼内常见的恶性肿瘤。随着现代科学技术的进步以及新的诊疗手段的问世，使得临床医生对于RB的治疗有了更多选择，也使得我们对于RB患儿选择采用不同的个体化综合治疗方案的治疗策略得以实施。传统的治疗理念在不断发生更新和转变，我们应根据我国RB发病的实际情况，学习借鉴国外对RB先进的治疗经验，努力探索并逐步建立符合我国国情的规范化RB综合治疗新模式，这对提高RB患儿生存率，改善生存质量都具有十分重要的意义。     

重视视网膜母细胞瘤发生机制和诊疗方法研究

视网膜母细胞瘤（RB）是致盲、致残、致死的最严重婴幼儿眼病。抑癌基因 RB1缺失导致肿瘤发生。随着治疗方法的不断创新和发展,RB治疗从眼球摘除、外放射治疗、静脉化学药物治疗、到眼动脉介入化学药物治疗,逐步实现了保生命、保眼球和保视力。但是,RB的发生机制仍存在很多空白点,眼内晚期患儿的眼球摘除率居高不下,治疗...     

视网膜母细胞瘤综合治疗后临床组织病理学特点及VEGF和Ki-67蛋白表达研究

目的 探讨视网膜母细胞瘤(RB)综合治疗后组织病理学和临床特点,以及综合治疗后RB的血管内皮细胞生长因子(VEGF)、Ki-67蛋白的表达情况。方法 对9例综合治疗后行眼球摘除术的病例的组织病理学和临床资料进行回顾性分析。使用免疫组织化学和实时多聚合酶链反应方法测量VEGF的表达,用免疫组织化学法测Ki-67的表达。结果 在9例眼球中,6例为RB国际分期D期的肿瘤,3例为E期。眼球摘除的原因为：肿瘤广泛玻璃体种植8例,肿瘤复发3例,大量视网膜下积液,肿瘤视网膜下种植3例,玻璃体出血2例,完全的牵引性视网膜脱离3例。在综合治疗期间肿瘤都表现为缩小,基底部宽度平均缩小43.7％,厚度平均缩小57.9％。3例表现为奶酪-芝士状消退模式,1例表现为鱼肉状消退模式,5例表现为混合型消退模式。计划性眼球摘除病例的VEGF表达低于复发性RB。结论 综合治疗后眼球摘除的最主要原因为广泛玻璃体种植。混合型消退模式是较常见的消退模式。综合治疗过程中行计划性眼球摘除病例VEGF表达降低。VEGF表达增高在综合治疗后RB的复发中可能起到一定的作用。     

视网膜母细胞瘤治疗中应注意的若干问题

个体化的综合治疗开创了视网膜母细胞瘤（RB）临床诊治的新纪元,国内数家眼科中心为此进行了有益的尝试,但从总体上对这一疾病的认识尚有待提高。本文就推广RB的国际分期、改变对RB认识上的误区、提高RB的早期诊断率、加强多中心协作,提高国内RB临床研究的质量、开展RB临床基础研究等五个方面做一简要述评。     

视网膜母细胞瘤基因治疗研究进展

视网膜母细胞瘤（RB）是婴幼儿最常见的眼内恶性肿瘤,传统的治疗方法主要采取眼球或眼眶内容物摘除术、放射疗法及化学疗法,但均不能明显改善预后,而RB的远期影响往往带来局部的破坏性后果。基因疗法为RB的治疗及预后的改善带来了契机,具有传统方法不可比拟的优势。就基因转染载体、基因导人方式、常用的基因技术（如RNA干扰技术）和治疗靶点等方面对RB基因治疗的研究进展进行综述。     

RB1基因检测在视网膜母细胞瘤诊疗中的应用

视网膜母细胞瘤(retinoblastoma,RB)是婴幼儿最常见的原发性眼内恶性肿瘤,其发生和发展与肿瘤抑制基因RB1的失活密切相关。对RB1基因检测进行深入研究,有助于在临床工作中选择最优化的策略开展基因检测和遗传咨询。RB严重危害患儿的生命及视功能,目前亟需提高我国RB患者基因检测和遗传咨询覆盖率。RB1基因检测有助于实现RB患者的个体化综合治疗和遗传指导,需要推进其在RB临床诊疗工作中的应用。     

视网膜母细胞瘤治疗及研究进展

随着基础研究和临床实践的逐步深入,视网膜母细胞瘤（ RB）的治疗模式已发生改变。目前,RB的治疗方法包括局部治疗、化学治疗、放射治疗、手术治疗、基因治疗等,本文将就RB的治疗及研究进展进行综述。     

视网膜母细胞瘤综合治疗的预后及其相关因素

目的：研究视网膜母细胞瘤（RB）患儿综合治疗的预后并探讨其相关临床因素。 方法：回顾性系列病例研究。分析在深圳市眼科医院进行综合治疗的65例（92眼）RB患儿的临床资料。综合治疗的方法为以化疗为主联合局部治疗（光凝或冷冻）+眼球摘除治疗。不同结局相关临床因素的比较采用独立样本t检验,其余指标采用Fisher's精准检验。 结果：所有RB患儿的总生存率为92%,不同分期生存率差异无统计学意义。所有患儿总的保眼率为51%,E期和其他4期之问保眼率差异均有统计学意义(P<0.01)。高风险D、E期RB患儿共60例,采用化疗联合局部治疗保眼的共42例,D期18例中11例保眼成功,E期24例中4例保眼成功。治疗成功与失败患儿的初诊时间和随访时间差异无统计学意义。根据眼内肿瘤的位置将42例患儿分为3组,分析显示即周边部（包括中周部）与后极部、后极部+周边部保眼率差异有统计学意义(P=0.009、0.021)。42例患儿中4例死亡,均为肿瘤位置处于后极部的患儿,后极部与后极部+周边部2组之间病死率差异有统计学意义( P=0.045)。 结论：综合治疗提高了RB患儿的生存率,但患儿的生存率和保眼率与其临床分期及肿瘤所处的位置高度相关。     

综合治疗前后视网膜母细胞瘤微血管密度和血管内皮生长因子表达研究

目的 观察视网膜母细胞瘤(RB)未行综合治疗和行综合治疗后微血管密度(MVD)、血管内皮生长因子(VEGF)表达以及与RB肿瘤视神经浸润和综合治疗后复发的关系.方法 61例RB石蜡标本纳入研究.分为未行综合治疗直接摘除眼球(未治疗)组、综合治疗前计划眼球摘除(计划眼摘)组、综合治疗后肿瘤复发(复发)组,3组分别为52、...     

眼脉络膜恶性黑色素瘤治疗的进展

对脉络膜恶性黑色素瘤（ｃｈｏｒｏｉｄａｌｍａｌｉｇｎａｎｔｍｅｌａｎｏｍａ，ＣＭＭ）的治疗，眼球摘除已不是唯一的治疗手段。光凝治疗作为主要治疗手段对小脉络膜恶性黑色素瘤有效，它也可作为放射治疗等的辅助治疗。国外放射治疗应用广泛。没有发现保留眼球的方法较眼球摘除更易发展成转移性疾病。综合治疗有更多的优点。     

角膜及眼表疾病研究中存在的问题及建议

我国在角膜及眼表疾病研究领域形成了自己的特色和优势，但在某些方面与国际学术水平相比仍有差距，本文就当前研究中应该重视的若干问题提出建议。加强感染性角膜病的流行病学和药物敏感性筛选研究，面向基层普及感染性角膜炎的正确治疗观念。重视眼表重建手术的临床和基础研究，提高对常见眼表疾病的诊断和治疗水平。加强具有原创性的基础研究，力求在生物组织工程、基因诊断和治疗、新型药物研制等方面取得进展，为角膜病专业学科水平的可持续发展提供强大动力。     

从R-E到IRC分级:视网膜母细胞瘤治疗观念的转变

目前我国大部分地区对于视网膜母细胞瘤仍采取手术摘除眼球的方法,虽然能提高一部分患儿的生存率,但手术不仅造成患儿生理上的创伤,对儿童将来的健康发展也有深远的心理影响。随着临床研究和治疗技术水平的提高,对患儿生活质量要求也不断提高。眼球摘除、外定向放疗(EBRT)等已不能满足这种要求,化疗联合局部治疗是目前国际上公认的首要治疗方法。同时,对放疗具有指导意义的R-E分级已不适合指导化疗,近年来提出的建立在化疗联合局部治疗基础上的IRC分级,具有非常重要的临床指导意义。(眼科,2007,16:368-372)     

Distant metastatic retinoblastoma without central nervous system involvement

Retinoblastoma is the most common intraocular malignancy in children, with a reported incidence ranging from 1 in 15,000 to 1 in 18,000 live births. Metastatic retinoblastoma is rare in developed countries, with a reported range from 4.8% in the United States to 5.8% in the United Kingdom. However, the frequency reported from developing countries varies from 9 to 11% at presentation. The mortality is very high owing to late presentations, delayed diagnosis compounded by socio-economic factors. The management of metastatic retinoblastoma is evolving, but it is still a challenge in pediatric oncology. We present a case of an extensive skeletal metastasis that initially presented as a massive orbital retinoblastoma.     

视网膜母细胞瘤的治疗新进展

视网膜母细胞瘤(retinoblastoma,RB)是儿童最常见的眼内恶性肿瘤,预后较差,严重影响患儿视力甚至危及生命。临床表现主要为白瞳症,发病机制普遍认为是Rb基因的二次突变。治疗RB需按个体化的原则,并结合其病理分期。国内外就该病的治疗,包括眼球摘除术、放射疗法、化学疗法、药物疗法、激光光凝疗法、光动力疗法、温热疗法、冷冻疗法、基因疗法等已取得了一定的进展,本文现综述如下。     

视网膜母细胞瘤药物治疗进展

在视网膜母细胞瘤的综合治疗中,化学减容（chemoreduction）是一项非常有效且不可替代的方法。但这些化疗药物存在潜在的毒副作用且对中晚期视网膜母细胞瘤的疗效不佳,因此如何减少药物副作用、增强疗效、减少耐药性成为近年来视网膜母细胞瘤研究的热点。研究集中于给药方法的变革和新型药物的研制,这包括眼动脉介入给药,纤维蛋白黏合剂缓释给药,药物直流电离子导入等,一些新的治疗药物的应用,如MDM2抑制剂（nutlins）、吩恶嗪衍生物Phx-1、考布他汀A4磷酸盐（combretastatin A4 phosphate,CA4P）、2-脱氧-d-葡萄糖（2-deoxy-d-glucose,2-DG）、组蛋白去乙酰化酶抑制剂（HDACi,包括TSA、HASA、MS．275、BL1521）等。本文将从这两个方面对近几年视网膜母细胞瘤的药物使用和研究进展作一综述。     

EDWARD COTLIER AND ROBERT WEINREB, EDITORS Retinoblastoma in Transgenic Mice: Models of Hereditary Retinoblastoma

Retinoblastoma, the most common intraocular malignancy in childhood, has served as a paradigm for the study of genetic mechanisms of oncogenesis. The retinoblastoma susceptibility gene RB1 was the first tumor suppressor gene to be cloned, and genetic and molecular biologic studies of this tumor have greatly expanded the understanding of the mechanics of tumorigenesis. Human retinoblastoma has essentially no naturally occurring animal counterpart. The development of transgenic murine models of retinoblastoma have created an experimental tool for manipulation of a tumor gene system in vivo. These models have also enabled studies of new therapeutic modalities. This review outlines the development of the transgenic murine models of retinoblastoma, together with the genetic mechanisms of retinoblastoma origin. Current therapeutic innovations developed by means of the transgenic models are described.     

Retinoblastoma in transgenic mice: models of hereditary retinoblastoma.

Retinoblastoma, the most common intraocular malignancy ill childhood, has served as a paradigm for the study of genetic mechanisms of oncogenesis. The retinoblastoma susceptibility gene RB1 was the first tumor suppressor gene to be cloned, and genetic and molecular biologic studies of this tumor have greatly expanded the understanding of the mechanics of tumorigenesis. Human retinoblastoma has essentially no naturally occuring animal counterpart. The development of transgenic murine models of retinoblastoma have created an experimental tool for manipulation of a tumor gene system in vivo. These models have also enabled studies of new therapeutic modalities. This review outlines the development of the transgenic murine models of retinoblastoma, together with the genetic mechanisms of retinoblastoma origin. Current therapeutic innovations developed by means of the transgenic models are described.     

“Retinoblastoma survival disparity”: The expanding horizon in developing countries

The patients’ survival for the most common intraocular tumor in children, retinoblastoma, has a wide spectrum among the world countries. This study was conducted to provide an overview of the retinoblastoma survival disparity worldwide by discussing the trends of patients’ survival, as well as recent advances in the management of retinoblastoma.The design of this study was literature review and commentary. Selected articles from PubMed (except one) including both developing and developed countries regarding the patients’ survival in retinoblastoma were considered and reviewed critically. An analysis of 47 articles was performed. In conclusion, in spite of an obvious contrast of retinoblastoma survival disparity in the world, the expanding horizons in developing countries are promising and continuing on all fronts and results are hope-inspiring.     

Second Nonocular Tumors in Retinoblastoma Survivors: Are They Radiation-induced?

A review of 693 patients with bilateral retinoblastoma and 18 patients with unilateral/germinal retinoblastoma was carried out to find the incidence, time, course and pattern of second nonocular tumors in retinoblastoma survivors. Of 688 patients who survived therapeutic radiation for retinoblastoma, 89 developed second tumors: 62 in the field of radiation and 27 out of the field. Of 23 patients who received no radiation, five developed second tumors: one in the field and four out of the field. The most common tumor found was a sarcoma both in and out of the field of radiation. The incidence of second tumors increases with time, although the mean latent period is 10.4 years. At 10 years, the incidence of second tumors is 20%, at 20 years, it is 50% and at 30 years, 90%. The incidence of tumors in patients treated without radiation and where tumors developed outside the field was 10% at 10 years, 30% at 20 years and 68% at 32 years. There was no relationship between incidence of tumors and dose of therapeutic radiation when analyzed with life tables. A second course of radiation therapy did not increase the incidence of second nonocular tumors.