Combined intra-arterial chemotherapy and intravitreal melphalan for the treatment of advanced unilateral retinoblastoma

●AIM:To evaluate the efficacy and safety of combined intraarterial chemotherapy(IAC)and intravitreal melphalan(IVM)for the treatment of advanced unilateral retinoblastoma.●METHODS:This retrospective study involved 30 consecutive eyes from 30 Chinese patients with advanced unilateral retinoblastoma.All patients were initially treated with IAC combined with IVM.The clinical status and complications were recorded at each visit.●RESULTS:The International Intraocular Retinoblastoma Classification groups were D in 23 eyes and E in 7 eyes.All eyes showed severe cloud vitreous seeds at the first visit.The mean number of IAC cycles and intravitreal injections was 3.2(range,3-4)and 6(range,1-14),respectively.The median follow-up time was 29 mo(range,7-36 mo).Treatment success with regression of the retinal tumor and vitreous seeds was achieved in 29 of 30 eyes(96.7%).Globe salvage was attained in 93.3%(28/30)eyes,and enucleation(n=2)was per formed due to neovascular glaucoma and persistent vitreous hemorrhage.Complications included retinal pigment epithelium(RPE)atrophy(n=13;43%),mild lens opacity(n=7;23%),vitreous hemorrhage(n=5;17%)and rhegmatogenous retinal detachment(n=1;3%).No extraocular tumor extension or metastasis occurred.●CONCLUSION:Combined IAC and IVM is effective and safe for the treatment of advanced unilateral retinoblastoma.

A delayed diagnosis of unsuspected retinoblastoma in an in vitro fertilisation infant with retinopathy of prematurity

Dear Editor,Iam Dr.Tian Tian,from the Department of Ophthalmology,Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai,China.I write to present a rare case report of a delayed diagnosis of unsuspected retinoblastoma(RB)in an in vitro fertilisation(IVF)infant with retinopathy of prematurity.The simultaneous presentation of RB and prematurity of retinopathy(ROP)in an IVF infant is very rare.The relationship between RB and IVF is still indeterminate and mechanisms that lead to ocular or systemic

An international survey of classification and treatment choices for group D retinoblastoma

AIM: To determine which IIRC scheme was used by retinoblastoma centers worldwide and the percentage of D eyes treated primarily with enucleation versus globe salvaging therapies as well as to correlate trends in treatment choice to IIRC version used and geographic region.METHODS: An anonymized electronic survey was offered to 115 physicians at 39 retinoblastoma centers worldwide asking about IIRC classification schemes and treatment patterns used between 2008 and 2012. Participants were asked to record which version of the IIRC was used for classification, how many group D eyes were diagnosed, and how many eyes were treated with enucleation versus globe salvaging therapies. Averages of eyes per treatment modality were calculated and stratified by both IIRC version and geographic region. Statistical significance was determined by Chi-square, ANOVA and Kruskal-Wallis tests using Prism.RESULTS: The survey was completed by 29% of physicians invited to participate. Totally 1807 D eyes were diagnosed. Regarding IIRC system, 27% of centers used the Children's Hospital of Los Angeles(CHLA) version, 33% used the Children's Oncology Group(COG) version, 23% used the Philadelphia version, and 17% were unsure. The rate for primary enucleation varied between 0 and 100% and the mean was 29%. By IIRC version, primary enucleation rates were: Philadelphia, 8%; COG, 34%; and CHLA, 37%. By geographic region, primary enucleation rates were: Latin America, 57%; Asia, 40%; Europe, 36%; Africa, 10%, US, 8%; and Middle East, 8%. However, systemic chemoreduction was used more often than enucleation in all regions except Latin America with a mean of 57% per center(P<0.0001). CONCLUSION: Worldwide there is no consensus on which IIRC version is used, systemic chemoreduction was the most frequently used initial treatment during the study period followed by enucleation and primary treatment modality, especially enucleation, varied greatly with regards to IIRC version used and geographic region.

Pathologic comparisons of enucleated eyes with retinoblastoma after superselective ophthalmic arterial chemotherapy with or without intravenous chemotherapy

AIM:To describe and compare pathologic findings in eyes enucleated after superselective ophthalmic arterial chemotherapy(SOAC)or SOAC with intravenous chemotherapy(IVC)for retinoblastoma.METHODS:Medical records between January 1st,2014 and June 30th,2017 were retrospectively analyzed,and pathologic findings were recorded.This study included 36 eyes from 22(61.1%)male and 14(38.9%)female patients.Nineteen of 36(52.8%)eyes received SOAC(mean=3,range=1-7)as primary treatment,and 17 of 36(47.2%)eyes received SOAC(mean=3.7,range=1-10)after IVC(mean=6.1,range=2-11).Tumor extension including choroidal invasion(n=9,25%),optic nerve invasion(n=5,13.9%)and anterior segment invasion(n=5,13.9%)were recorded.RESULTS:Histopathologic evidence of ischemic damage in the retina and choroid was found in 28(77.8%)eyes.Thrombosed blood vessels were identified in 9(25%)eyes,including orbital artery in the retrobulbar orbit(n=1),intrascleral vessels(n=4),and chorioretinal vessels(n=6).Fibrotic changes were found in extraocular muscles(n=5,13.9%)and optic nerve(n=5,13.9%).Varying degrees of scleral degeneration were found in all eyes.In statistical analysis,there was no significant difference in clinical and pathologic changes between SOAC group and SOAC with IVC group except for optic nerve invasion(P=0.047).CONCLUSION:SOAC for retinoblastoma can result in ocular toxicity,and SOAC with IVC do not increase the toxicity but reduced the incidence of optic nerve invasion.