视网膜母细胞瘤术后存活20年以上的随访观察

为探讨视网膜母细胞瘤（ＲＢ）患者长期存活的有关因素及第二恶性肿瘤发病及遗传性，对１９６０—１９７５年病理确诊的９０例ＲＢ患者及其后代进行随访。随访到５９例，其中存活２０年以上者１６例，存活率为２７．１２％。尚未发现发生第二恶性肿瘤者。随访到ＲＢ患者的后代１４人，除２人因腹泻死亡外，其余经检查均未发现ＲＢ病变。结论：长期存活与病程、病期及是否做放疗有关，未分化型者的预后并不差，只要早期发现，及时治疗仍可长期存活。     

30例视网膜母细胞瘤术后放疗的长期随访

我们自1991年5月至1996年5月共收治42例(48眼)视网膜母细胞瘤(retinoblastoma,RB)术后患者,采用放射治疗.现将有完整资料的30例报告如下. 1 资料与方法 30例患者,男17例,女13例,5岁以下占96.7%.30例为眼外期,10例为眼内期,2例分期不明.30例眼外期RB患者中,28例行单眼或双眼眼球摘除术,术后病理证实为眼外期视网膜母细胞瘤,且12例患者术后病理证实视神经断端及眶内有残留,术后2周放置义眼并开始行体外放射治疗.照射方法视病变范围而定.用前野和颞侧野照射,颞侧野照射要注意保护晶状体.侵及双眼的患者照射时注意尽可能保存未摘除眼的视力.放疗剂量DT:50～60 Gy/5～7周.全组均随访5年以上.     

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。     

化疗缩小双侧视网膜母细胞瘤

目的：评价应用化疗和局部治疗双侧视网膜母细胞瘤的方法。     

视网膜母细胞瘤扫描电镜的组织形态学观察

从十六世纪中叶起,病理学家就开始对视网膜母细胞瘤(Retinablastoma)的组织病理学特征及其来源进行了研究。1864年Virehow(?)认为该肿瘤来自神经胶质细胞,故命名为神经胶质瘤。到1926年Verhoeff则认为它来自光感受细胞,命名为视网膜母细胞瘤,而延用至今。1981年有人通过组化染色方法进行     

视网膜母细胞瘤的病理改变与预后

从病理学角度对视网膜母细胞瘤的光镜、电镜、免疫组织化学和分子遗传学等方面简要阐述,并将这些病变特征与预后之间关系逐一分析,提出可能影响视网膜母细胞瘤患者生存时间的因素。     

高危视网膜母细胞瘤患者的术后辅助化疗

目的探讨术后辅助化疗对高危视网膜母细胞瘤（Rb）患者生存及预后的影响。方法收集1997～2004年于中山眼科中心诊治的268例Rb患者资料,随访了135例,其中男80例,女55例;单眼107例,双眼28例。分析患者的一般状况、病理结果、治疗情况、生存率等。用SPSS13.0软件包进行统计学分析。结果135例患者中经治疗但未行化疗75例,曾行化疗51例,未行治疗9例;存活111例（其中1例双眼患者的未摘除眼肿瘤仍有进展）,死亡24例,主要死亡原因为肿瘤颅内蔓延。51例化疗患者中存活46例,死亡5例。未经化疗的患者总体生存率略低于经过化疗的患者,两者间无统计学差异（P=0.369）。眼外期患者中化疗组生存率明显高于未经化疗组,两者间有统计学差异（P=0.000）。未行化疗的患者与经过化疗的患者Ⅰ期义眼座植入后暴露率无统计学差异（P=0.815）。结论术后辅助化疗对有复发和转移高危因素的视网膜母细胞瘤患者可提高其生存和预后,多数患者可耐受化疗的副作用,建议对此类患者术后辅以化疗。     

视网膜母细胞瘤眼球摘除术后病理与预后分析

目的　分析视网膜母细胞瘤（ｒｅｔｉｎｏｂｌａｓｔｏｍａ,Ｒｂ）侵犯眼球部位与患者预后之间的关系,为病情的预测和治疗提供依据。方法　回顾性分析２００３年１月至２０１１年２月于中山大学中山眼科中心行眼球摘除术的单眼Ｒｂ患者。记录术后肿瘤侵犯的部位、治疗情况和生存情况并评估患者的５ａ生存率（５ｙｅａｒｐｒｏｂａｂｉｌｉｔｙｅｖｅｎｔ-ｆｒｅｅｓｕｒｖｉｖａｌ,５-ＰＥＦＳ）。随访以患者死亡或至２０１２年２月截止,随访时间为（４６．３±２９．２）个月。结果　共有２０２例２０２眼患者纳入研究,其中筛板及筛板前侵犯所占比例最大（４０．６％）,５-ＰＥＦＳ也最高（９６．１％）;筛板后视神经侵犯和视神经断端侵犯者５-ＰＥＦＳ分别是８２．５％和４０．０％,三者比较差异有显著统计学意义（Ｐ＜０．０１）。筛板后视神经侵犯接受治疗的患者５-ＰＥＦＳ（９１．６％）有高于未治疗者（６６．７％）的趋势（Ｐ＝０．０９）;视神经断端侵犯者接受治疗与未治疗的５-ＰＥＦＳ分别是５０．０％和０（Ｐ＜０．０１）,单纯脉络膜侵犯患者５-ＰＥＦＳ是９３．８％,巩膜侵犯患者接受和未接受辅助化疗的５-ＰＥＦＳ分别是５７．１％ 和７７．８％（Ｐ＞０．０５）。２例眼眶侵犯患者均死亡,４例患者眼球摘除术后眼眶复发,从眼球摘除术到眼眶复发的时间是５～１５个月,平均为９．８个月。结论　Ｒｂ眼球摘除术后病理检查对患者预后的预测和辅助治疗有指导意义;眼球摘除术后２ａ,尤其１ａ内是Ｒｂ复发的高发期,这期间所有患者需要密切随诊。     

视网膜母细胞瘤术后23 a未复发1例

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,恶性程度高,预后很差.眼球摘除术是目前常采用的治疗方法,术后辅助治疗(化疗、放疗)及长期的随访观察与其预后密切相关,现将1例术后随访23 a未复发的患者情况报告如下.     

Glaucoma in Retinoblastoma

The association between retinoblastoma and secondary glaucoma is well known. The most common cause of secondary glaucoma in retinoblastoma is iris neovascularization (NVI) followed by pupillary block and tumor seeding of the anterior chamber. Although glaucoma is a secondary clinical issue in retinoblastoma (RB) management and care, awareness of its presence, revealed by a thorough ocular exam of the anterior segment, can guide the clinician in assessing the overall condition of the affected eye.     

Retinoblastoma associated orbital cellulitis

AIM—Preseptal and orbital cellulitis are rare presenting features of intraocular retinoblastoma. The objectives of this study were to determine the frequency of retinoblastoma associated cellulitis, as well as to review its clinical and histopathological features.
METHODS—The medical records of 292 retinoblastoma patients in the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia were reviewed. Those indicating a history of, or presenting with, cellulitis were retrieved and their clinical, radiological, and histopathological variables were assessed. Patients with definite extraocular tumour extension on clinical or radiological examination were excluded.
RESULTS—14 patients were found to have retinoblastoma associated cellulitis (4.8%); nine had bilateral and five had unilateral retinoblastoma. Conjunctival and blood cultures were performed in 10 cases and were negative. 10 children were treated with intravenous steroids, often in conjunction with antibiotics, resulting in a prompt decrease in inflammation. Three other children were treated with antibiotics alone and one received no treatment. Computed tomographic scanning depicted large intraocular tumours occupying between 80% and 100% of the globe in each case. In eight patients, periocular inflammation was radiologically interpreted as possible extraocular extension. In one patient serial computed tomographic scanning showed a reduction in intraocular calcification over time which occurred in the presence of cellulitis. 12 patients underwent enucleation and histopathological examination revealed large necrotic, poorly differentiated tumours associated with uveal involvement and early optic nerve invasion. Focal perilimbal destruction was seen in one patient, and in another peripapillary extrascleral extension was present. 12 patients are alive with a mean follow up of 56.4 months.
CONCLUSIONS—Radiological evaluation of scleral integrity may be hindered by periocular inflammatory changes. The orbital cellulitis correlated well with the presence of advanced intraocular retinoblastoma with massive necrosis and anterior chamber involvement. In the majority of patients, cellulitis was not indicative of an extension of retinoblastoma into the orbit. Intravenous steroid treatment reduced orbital inflammation, facilitating examination and subsequent enucleation.

Keywords: retinoblastoma; orbital cellulitis     

视网膜母细胞瘤的组织培养

将临床诊断为RB 病人的眼球标本,取玻璃体肿瘤细胞和肿瘤组织块分别置199—1640混合培养液,37℃恒温培养。玻璃体肿瘤细胞呈单个悬浮生长,细胞单一,不易结成团块。肿瘤组织块培养的肿瘤细胞从组织块边上移行出来,脱落到培养液内呈团块状悬浮生长。二者的肿瘤细胞增殖均按延迟期、生长期和静止期这样一个周期生长,大约需3—4周。肿瘤组织块培养的瘤细胞增殖情况和细胞形态,较玻璃体肿瘤细胞为好。     

Hereditary Diffuse Infiltrating Retinoblastoma

Retinoblastoma is one of the most common childhood cancers. The diffuse infiltrating retinoblastoma is a rare subtype of this neoplasm. The majority of cases of diffuse infiltrating retinoblastoma are unilateral and occur sporadically. Herein we report on a family with three children affected by retinoblastoma, among them one girl with diffuse infiltrating retinoblastoma. This girl was diagnosed at the age of 8 years with a unilateral diffuse infiltrating retinoblastoma. By contrast, the two brothers became clinically apparent in the first 2 years of life with bilateral retinoblastoma. The parents were clinically unremarkable. Genetic analysis of RB1 gene was performed. The girl with diffuse infiltrating RB was found to be heterozygous for an oncogenic mutation in the RB1 gene that was also carried by both brothers and the father of the family. These results show that diffuse infiltrating retinoblastoma can develop on the background of a hereditary predisposition to retinoblastoma.     

Gene Carrier Detection in Retinoblastoma

Although the presence of the retinoblastoma (RB) gene is usually made obvious by bilateral RB tumors, unaffected relatives of RB patients and unilateral RB patients may also carry the gene. Patients with 13q14 deletion have RB, and segregation of RB with markers (Q-banding and Esterase D) on chromosome 13 can be studied in some two-generation RB families. Radiosensitivity of fibroblasts may be a marker or may be present only in chromosome 13 deletion patients. Growth properties of fibroblasts suggest that the RB gene itself is expressed in normal cells. Ultimately, gene-carrier detection may be achieved by defining gene expression, gene product, or by cloning the gene itself.     

视网膜母细胞瘤的临床病理及预后

视网膜母细胞瘤是儿童常见的眼内恶性肿瘤，严重威胁生命。本文通过对５５例视网膜母细胞瘤患２－２２上的随访，对其性别，发病年龄、病程、临床分期，组织病理学及治疗等进行了统计学分析，探讨了影响预后的有关因素。     

Observation of Surface Structure of Retinoblastoma Cell Line

Objective: We study the adhesion moleclues on the surface of SO-Rb50 retinoblastoma cell line.Motheds : The distribution of proteoglycans in the retinoblastoma SO-Rb50 cell line was analyzed by histochem-electron microscopy, using Colloidal Iron in combination with a series of enzyme digestions. In addition, immunohistochemistry was performed using a panel of specific antibodies including neuron specific enolase(NSE) ,glial fibrillary acidic protein(GFAP) , S-100 protein, fibronectin, laminin, and collagen IV.Results: Immunohistochemical stains showed the most marked cytoplasmic reactivity of SO-Rb50 cells with anti-NSE and anti-S100. The cells member and surface was postive with anti-NSE. No reactivity was noted with antibodies against laminin, GFAP, and collagen IV. After incubated with colloidal iron solution, three types of colloidal iron-positive stained material could be distinguished based on difiierencens in shape, size, electron density: (1) electron dense particles, (2) the larger colloidal ir     

Retinoblastoma: postenucleation orbital recurrence

Objective: The objective of this study was (i) to identify clinicopathological variables that significantly influence the frequency of postenucleation orbital recurrence and (ii) to create an awareness regarding follow-up, to pre-empt orbital recurrence.Design: Retrospective, case-controlled, longitudinal study.Participants: A total of 176 children with retinoblastoma who were admitted and treated at Khyber Teaching Hospital between August 1, 1978, and July 31, 1998.Methods: This study was undertaken by reviewing the appropriate charts. The data of children with primary enucleation were analyzed to extract cases of orbital recurrence.Results: The mean age was 3.8 years (range 1–10 years). Of 176 patients, 83 (47.3%) presented with proptosis and fungating mass. Of 136 patients who were enucleated, 80 (58.82%) presented with proptosis and fungating mass. The 31 patients who had been diagnosed as having had orbital recurrence had a mean age of 4.09 years. The duration between enucleation and orbital recurrence was 1–12 months in 28 patients (90.4%). Histopathology reports of only 8 patients (26%) could be traced. Follow-up was recorded in only 9.6% of cases.Conclusions: The failure to identify clinical and pathological parameters in the course of the disease in retinoblastoma children results in human misery caused by poor follow-up and gross negligence.     

Orbital Retinoblastoma in an Adult

ABSTRACT

Purpose: Retinoblastoma is usually seen in children before 5 years of age. We report an unusual case of retinoblastoma in an adult who presented to us with an orbital mass.

Methods: A 24 year-old-male presented to our centre with a history of protrusion of the right eye for 6 months, and associated loss of vision. Ultrasonography B-scan revealed an intraocular mass with calcification and MRI of the orbits showed extra-ocular spread. An incisional biopsy was taken from the orbital mass.

Results: On biopsy, histopathologic features and immunohistochemical stains were consistent with retinoblastoma.

Conclusion: To our knowledge, this is the first report of retinoblastoma presenting as an orbital mass in adulthood and highlights the importance of considering this tumour in the differential diagnosis of an intraocular mass with orbital extension in an adult patient.     

视网膜母细胞瘤并发白内障的手术效果

目的：分析视网膜母细胞瘤（Rb）患儿并发白内障的诊断时间、手术时间,手术方式以及手术效果,并评估其行白内障手术的时机和安全性。方法：回顾性分析2010年1月至2016年12月在北京儿童医院、北京同仁医院和泉州市儿童医院行白内障摘除术的Rb患儿23例（25眼）。其中男11例（11眼）,女12例（14眼）。7眼行透明角膜25G切口,18眼行睫状体平坦部25G或者27G巩膜微切口。随访1～72个月,平均（24±16）个月。统计手术时间,术前Rb治疗方式及次数,术后眼底检查是否有肿瘤复发或转移。结果： 患儿平均手术年龄（35±19）个月。自确诊Rb到发现白内障的时间（中位数）为19 个月,发现白内障到白内障手术时间（中位数）为5个月,最后一次Rb治疗到行白内障手术的时间（中位数）是10个月。行白内障摘除术前全身化疗22例（102次）,局部光凝12眼（62次）,眼内注药11眼（19次）,放疗1眼（1次）,眼外冷冻6眼（10次）,玻璃体切割手术17眼（20次）。2眼在Rb治疗过程中不能查见眼底,而眼B超检查示肿瘤复发,故行白内障手术,术后局部激光治疗复发肿瘤,随访期内未再复发。23眼为肿瘤局限钙化后行白内障手术,术后19眼无肿瘤复发及转移,未做治疗;4眼出现肿瘤晚期并发症（1眼继发青光眼,2 眼前房出血,1 眼眼内肿瘤复发）,行眼球摘除术。术后行病理检查,摘除眼未见眼球外有Rb转移。结论：对于Rb患儿,必要的白内障手术为眼底检查提供了清晰的视野。对于肿瘤局限钙化病情稳定的患眼,在无肿瘤生长的角膜或者睫状体平坦部做切口行白内障手术是安全的。