Subretinal Seeds Research Articles

Ruthenium-106 (106Ru) plaque brachytherapy as salvage treatment for retinoblastoma following intravenous chemotherapy

PURPOSETo describe the clinical presentation and treatment outcomes of patients undergoing Ruthenium-106 (106Ru) plaque brachytherapy as salvage treatment for retinoblastoma (RB) following intravenous chemotherapy (IVC). METHODSRetrospective chart review of 44 eyes of 42 patients. The indications for plaque brachytherapy included solid tumor recurrence (n=20; 45%), solid tumor residual (n=16; 36%), new subretinal seeds (n=5; 12%), and new solid tumor (n=3; 7%). RESULTSThe median age at the presentation was 12 months (range, 3–72 months). Based on ICRB classification, 8 (18%), 8 (18%), 16 (36%), and 5 (12%) tumors belonged to Groups B, C, D, and E, respectively. A median interval of 5 months (range 3–21 months) was noted between the last IVC cycle and plaque brachytherapy. The mean tumor height was four mm (range, 1.5–6 mm). All patients were treated with 106Ru plaque (round or notch) with a median total dose of 45 Gy (range, 40–55 Gy) delivered to the tumor apex. At a mean post plaque follow-up period of 28 months (median, 23 months; range, 3–132 months), tumor completely regressed in 25 eyes (56%). Tumor recurrence within the plaque site was noted in eight eyes (18%) associated with a type 2 regression pattern (75%). At the last follow-up, the globe salvage rate was 24 eyes (55%), while 2 patients (5%) died due to metastasis. CONCLUSION106RU plaque brachytherapy can be a useful salvage treatment for focal tumors (new or recurrent) following systemic IVC.

EPCO-05. GENOMIC AND CLINICAL INSIGHTS FROM A LARGE-SCALE STUDY OF 172 RETINOBLASTOMAS

Abstract Retinoblastoma, a pediatric ocular cancer, is the only central nervous system tumor visible without specialized tools, readily detectable with the naked eye. This rare childhood cancer is mainly driven by mutations in RB1 or MYCN amplification, posing significant challenges in pediatric oncology. We conducted whole exome sequencing on 172 retinoblastoma tumors along with matched blood DNA from 171 patients, which included 119 unilateral, 49 bilateral, and three trilateral cases. Our analysis identified pathogenic variants in RB1 in 76 patients and MYCN duplication in 79 cases, highlighting their crucial roles in the disease. Additionally, we found mutations in RPTOR and TSC2, components of the mTOR signaling pathway, in two patients. Notably, our copy number alteration analysis revealed recurrent arm-level changes, including gains on 1q (23.3%), 2p (20.9%), and especially 6p (41.3%), along with other changes such as 5p gain (8.1%), 17q gain (8.1%), and 16q loss (5.8%). Our findings link these genetic alterations to specific clinical features; for instance, 6p gains were associated with Rubeosis, subretinal seeds, and tumor LC beyond, while 17q gains were linked to Rubeosis. Furthermore, gene collapsing techniques identified MATR3 and CYP4F2 as additional novel retinoblastoma-related genes, and module identification analysis pinpointed retinoblastoma-associated modules including HLA-DRB1-CBL, LPAR2, RB1, and TRIM28. Our study, the largest cohort of its kind, underscores the importance of next-generation sequencing from understudied populations in uncovering the complex genetic landscape of retinoblastoma, emphasizing the potential of precision medicine to enhance therapeutic strategies and improve outcomes for retinoblastoma patients.

Outcome of 6 Cycles of Primary Intravenous Chemotherapy of Retinoblastoma Patients in Terms of Recurrence in a Tertiary Health Care Hospital

Objective: To determine the frequency of recurrence after 6 cycles of primary intravenous chemotherapy of retinoblastoma patients in a tertiary health care hospital.
 Materials and Methods: This study was conducted at the Department of Ophthalmology, Lahore General Hospital, Lahore from March 15, 2020 to September 15, 2020. A total of 81 patients were selected randomly from outpatient department. Performa designed was used to collect data including name, age, gender, registration number, laterality, age at diagnosis, group of disease, treatment given and outcomes of given treatment. International Intraocular Retinoblastoma Classification (IIRC) was used for classification of the disease. The chemotherapy regimen used was vincristine, etoposide and carboplatin (VEC). A total of 6 cycles were given at 3 weekly intervals. Serial monitoring of the tumors was done to rule out any local recurrence or side effects of therapy.
 Results: Total 81 children presenting with retinoblastoma were enrolled in this study. There were 52(64.2%) male and 29(35.8%) female. The mean age of patients was 3.41±1.45 year. Unilateral disease was in 61 (75.30%) patients while 20 (24.70%) patients had bilateral retinoblastoma. Among 81 children, 20(24.7%) had recurrence of the disease.
 Conclusion: Development of recurrence after intravenous chemotherapy was noted in 24.7 % of all retinoblastoma eyes. Younger patients with more advanced, posteriorly located tumors and subretinal seeds at presentation were at increased risk, but recurrence can often be managed with globe-sparing therapy.

SECONDARY SALVAGE INTRAVENOUS CHEMOTHERAPY FOR REFRACTORY/RECURRENT RETINOBLASTOMA: A Study of 41 Eyes.

To determine the efficacy of secondary salvage intravenous chemotherapy (IVC) for refractory/recurrent retinoblastoma. Retrospective, nonrandomized interventional case series of 41 eyes of 33 patients with recurrent retinoblastoma. Of the 33 patients, mean age at the time of commencement of salvage IVC was 5 years (median, 5 years; range, 2-8 years). At presentation, recurrent retinoblastoma in 41 eyes of 33 patients was classified by the International Classification of Retinoblastoma as Group B (n = 7; 17%), Group C (n = 3; 7%), Group D (n = 16; 39%), and Group E (n = 15; 37%). All patients received 6 cycles of IVC as primary treatment. The indication for secondary salvage IVC with focal treatment included recurrent solid tumor (n = 36; 88%), subretinal seeds (n = 22; 54%), or persistent solid tumor (n = 2; 5%). Mean number of cycles of salvage IVC were 8 (median, 6; range, 6-18). Over a mean follow-up period of 43 months (median, 43 months; range, 12-96 months) after completion of salvage IVC, globe salvage was achieved in 22 (54%) eyes, 1 (3%) patient had histopathology-proven bone metastasis, and 1 (3%) patient died because of presumed metastasis. Secondary salvage IVC with appropriate focal treatment allows globe salvage in 54% eyes with refractory/recurrent retinoblastoma and thus serves as an alternative to intraarterial chemotherapy or enucleation.

Retinoblastoma associated with total exudative retinal detachment: Treatment and outcomes.

To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma (RB) and total retinal detachment (RD). Retrospective study of 50 eyes in 43 patients. Mean age at presentation was 15 months (median, 9 months). RB associated total RD was unilateral (n=36; 84%) or bilateral (n=7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm) and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n=26; 52%), vitreous seeds (n=13; 26%), and subretinal exudation in (n=4; 8%). Based on 8th edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n=19; 38%), cT2b (n=28; 56%), or cT3c (n=3; 6%). Primary treatment included intravenous (n=48; 96%) or intra-arterial chemotherapy (n=2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7 to 62 months), RD resolved in 41 (82%) eyes, tumor recurrence was noted in 39 (78%) eyes, globe salvage was achieved in 35 (70%) eyes and one (2%) patient died due to metastasis. Relatively high tumor recurrence rate is noted in RB presenting with total RD. With appropriate treatment, 70% of the globes can be salvaged.

Follow-up of retinoblastoma using RetCam fluorescein angiography and correlation with clinical findings.

To report RetCam fluorescein angiographic (FA) changes and correlation with clinical findings in the follow-up of retinoblastoma (RB) after frontline intravenous chemotherapy and other treatments. Patients having RB who underwent post-treatment RetCam fundus photography and FA under general anesthesia between February 2020 and February 2022 were retrospectively analyzed. 78 eyes of 70 patients with RB were included. In 55 (70.5%) eyes with type 1, 2 or 3 regression patterns, the main tumor started to show hyperfluorescence in arterial phase in 24 eyes (43.6%), in early venous phase in 24 (43.6%) eyes, and in late venous phase in 7 (12.7%) eyes. Of thirty-six (46.2%) eyes with chorioretinal scars developing after focal treatments (cryotherapy and transpupillary thermotherapy), window defects and visible choroidal vessels were found in all eyes, scleral staining in 10 (27.8%), and gliosis on scar tissue in 8 (22.2%). Vitreous seeds showed hyperfluorescence if they were calcified (6 eyes, 7.7%) and hypofluorescence if non-calcified (11 eyes, 14.1%). Retrohyaloid seeds (11 eyes, 14.1%) were hypo/isofluorescent while subretinal seeds (6 eyes, 7.7%) were hyperfluorescent. Leakage from peripheral retinal vessels was detected in 14 (17.9%) eyes and non-perfusion in 6 (7.7%) eyes. RetCam wide-angle FA is useful to evaluate the tumor and associated vascular and retinal changes after treatment in RB. Regressed tumors demonstrate later fluorescein uptake in venous phases. Fluorescein angiographic changes in chorioretinal scars include window defects, visibility of choroidal vessels, scleral staining, and leakage from vessels. Retinal vascular leakage and peripheral non-perfusion can be seen in eyes with regressed stable tumors.

Primary Macular Retinoblastoma: Clinical Presentation and Treatment Outcomes.

Purpose: To describe the clinical features and treatment outcomes in eyes with primary macular retinoblastoma (RB). Methods: Patients with primary macular RB were analyzed. Results: Of 41 patients (47 eyes), 20 (49%) were boys and 21 (51%) were girls. The mean age at diagnosis was 16 months (range, 1-60 months). The RB was bilateral in 6 patients (15%). At presentation, the macula was completely covered with the tumor in 22 eyes (47%) and partially covered with the fovea spared in 13 eyes (28%) and the fovea involved 12 eyes (25%). Based on the International Classification of Intraocular Retinoblastoma, 25 (53%), 15 (32%), and 7 (15%) of tumors were in Group B, Group C, and Group D, respectively. The tumor had exophytic features in 36 eyes (77%). The mean tumor basal diameter was 10.0 mm and the mean thickness, 5.6 mm. Associated features included subretinal seeds (10 eyes; 21%) and surrounding subretinal fluid (16 eyes; 34%). Forty-three eyes (92%) were treated with intravenous chemotherapy, 2 (4%) with intra-arterial chemotherapy, and 2 (4%) with transpupillary thermotherapy. Local tumor control was achieved in 45 eyes (96%), with 33 eyes (70%) showing a type III regression pattern. Over a mean follow-up of 23 months (range, 3-48 months), the macular tumor recurred in 5 eyes (11%), the globe was salvaged in all eyes with associated foveal atrophy (36 eyes; 77%), and 1 patient (2%) died. Conclusions: Macular RB has a good prognosis for globe salvage while vision salvage might be compromised as a result of associated foveal atrophy.

Intravitreal chemotherapy in the management of retinoblastoma in a resource-limited setting

Intravitreal chemotherapy (IVitC) in the management of retinoblastoma has increased the rate of globe salvage, specifically in patients with recurrent disease and associated vitreous seeds. A significant number of children with retinoblastoma in developing countries present late, resulting in higher intraocular tumour-stage at presentation. Treatment requirements for such tumours usually include intravenous chemotherapy (IVC) and/or intra-arterial chemotherapy (IAC). While IVC has a long usage track record and a good efficacy, it has been reported to be associated with higher recurrence rates in a significant number of patients. Intra-arterial chemotherapy has the advantage of lower recurrence rates but requires personnel with advanced interventional radiology skills and has limited efficacy in treating intravitreal seeds. Intravitreal chemotherapy has gained popularity recently, largely because of its superior efficacy in the management of vitreous seeds, subretinal seeds and recurrent retinal tumour. An 8-month-old male infant initially presented with bilateral retinoblastoma, International Classification System for Intraocular Retinoblastoma (ICRB) Group E in the right eye and Group B on the left eye. The right eye was enucleated and currently has a prosthesis. The left eye had tumours that initially responded to brachytherapy and transpupillary thermotherapy (TTT). Approximately two years later his tumours recurred with vitreous seeds and were successfully managed with the use of cryotherapy and intravitreal chemotherapy. The simplicity of the technique of IVitC and its efficacy in controlling vitreous seeds and recurrent retinal tumours makes this route of regional chemotherapy a viable one in areas with limited expertise and resources such as South Africa.

Iodine-125 Plaque Radiotherapy for Retinoblastoma Recurrence Following Intra-arterial Chemotherapy.

To assess the efficacy and toxicity of Iodine-125 (I-125) plaque radiotherapy for retinoblastoma following intra-arterial chemotherapy (IAC). Clinical records of patients with retinoblastoma who received I-125 plaque radiotherapy after IAC at the Ocular Oncology Service at Wills Eye Hospital between December 1, 2009 and April 30, 2020, were retrospectively reviewed. Forty-one retinoblastomas in 41 eyes of 41 patients were treated with I-125 plaque radiotherapy after IAC at a median age of 32 months. The indication for plaque radiotherapy was solid tumor recurrence with or without overlying subretinal/vitreous seeds (n = 33, 80%), subretinal seeds alone (n = 6, 15%), and vitreous seeds alone (n = 2, 5%). The median irradiated basal diameter and thickness was 9 and 4 mm, respectively. Mean radiation dose to tumor apex was 3,483 centigray (cGy) delivered at mean rate of 35 cGy/hr. The irradiated site was controlled in 39 eyes (95%) at a median of 20 months after plaque radiotherapy for solid tumor (31 of 33, 94%), subretinal (6 of 6,100%), and vitreous seeds (2 of 2, 100%). A subgroup of tumors occurring within an ischemic retinal/choroidal field was identified on fluorescein angiography (n = 24) and demonstrated control in 22 of 24 (92%). Using Kaplan-Meier analysis, radiation complications at 2 years included vitreous hemorrhage (37%), retinopathy (28%), papillopathy (18%), and cataract (18%). Five eyes (12%) were enucleated for recurrence outside the irradiated area, chronic vitreous hemorrhage, and/or total retinal detachment. Iodine-125 plaque radiotherapy provided 95% control for retinoblastoma tumors that failed IAC, including those in ischemic fields untreatable with further chemotherapy. Radiation complications should be anticipated in eyes exposed to substantial chemotherapy. [J Pediatr Ophthalmol Strabismus. 2022;59(3):164-171.].

Resident Perspectives 56-5

Resident Perspectives 56-5

Retinoblastoma seeds: impact on American Joint Committee on Cancer clinical staging

AimTo investigate whether the American Joint Committee on Cancer (AJCC) clinical category cT2b needs to be subclassified by the type and distribution of retinoblastoma (RB) seeding.MethodsMulticentre, international registry-based data were...

Retinoblastoma genetics screening and clinical management

BackgroundIndia accounts for 20% of the global retinoblastoma (RB) burden. However, the existing data on RB1 gene germline mutations and its influence on clinical decisions is minimally explored.MethodsFifty children with RB underwent complete clinical examination and appropriate multidisciplinary management. Screening of germline RB1 gene mutations was performed through next-generation sequencing and Multiplex Ligation-dependent Probe Amplification (MLPA) analysis. The mutation and non-mutation groups were compared for clinical parameters especially severity, progression and recurrence.ResultsTwenty-nine patients had bilateral RB (BLRB) and 21 had unilateral RB (ULRB). The genetic analysis revealed 20 RB1 variations in 29 probands, inclusive of 3 novel mutations, known 16 mutations and heterozygous whole gene deletions. The mutation detection rate (MDR) was 86.2% in BLRB and 19% in ULRB. Associations of disease recurrence (p = 0.021), progression (p = 0.000) and higher percentage of optic nerve invasion, subretinal seeds and high-risk pathological factors were observed in the mutation group. Clinical management was influenced by the presence of germline mutations, particularly while deciding on enucleation, frequency of periodic follow up and radiotherapy.ConclusionsWe identified novel RB1 mutations, and our mutation detection rate was on par with the previous global studies. In our study, genetic results influenced clinical management and we suggest that it should be an essential and integral component of RB-care in India and elsewhere.

Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma: Risk factors and treatment results.

To evaluate the risk factors leading to recurrence and new tumor (NT) development in patients with retinoblastoma after intravenous chemotherapy (IVC) and to review the treatment outcomes. The records of 166 retinoblastoma cases (having 246 affected eyes) who underwent six-cycle IVC (vincristine, etoposide, and carboplatin) as primary treatment between October 1999 and August 2020 were reviewed retrospectively. The mean ages at presentation were 9.0 (median: 8.0) and 9.2 (median: 8.5) months in cases with recurrence and NTs respectively. Recurrence was detected in 40 (16.3%) eyes, NTs in 29 (11.8%), and both recurrence/NTs in 24 (9.8%). The mean time elapsed till recurrence and NT was 10.7 months. Multivariable analysis showed that the factors predictive of recurrence were largest tumor base diameter (LTBD) >12 mm (p = 0.039) and presence of subretinal seeds at diagnosis (p = 0.043). Multivariable risk factors for the development of NTs were bilateral familial retinoblastoma (p = 0.001) and presence of subretinal seeds at diagnosis (p = 0.010). Mean follow-up was 80.1 (median: 72.5) months. By Kaplan-Meier analysis, the 1-, 3-, and 6-year recurrence and NT rates were 21.2%, 28.1%, and 28.7% and 14.9%, 22.6%, and 23.9% respectively. The most common treatment methods used for recurrent and/or NTs included cryotherapy, transpupillary thermotherapy, and intra-arterial chemotherapy. Enucleation was eventually required in 24/93 (25.8%) eyes. No patient developed metastasis. Development of recurrence and/or NT after IVC was noted in 38% of all retinoblastoma eyes. Bilateral familial disease, LTBD >12 mm, and presence of subretinal seeds at baseline were risk factors for recurrence and NTs in this study.

Pathologic evidence of retinoblastoma seeds supported by field emission scanning electron microscopy and Raman spectroscopy.

Purpose:The aim of this study was to examine the pathology of retinoblastoma (RB) seeds with supportive evidence by field emission scanning electron microscopy and Raman spectroscopy.Methods:This study was a laboratory-based observational study. Enucleated eyeballs received in the ocular pathology department of a tertiary eye care center in northeast India were included in the cohort after obtaining written informed consent during the surgery. The study was carried out for 6 years (2015–2020). Most of the eyeballs were Group-E RBs. Standard eyeballs sectioning were done by bread loaf techniques. Gross documentations included RB seeds seen in the smallest calotte done with utmost care. Seeds were documented also in permanent sections. Scanning electron microscopy and Raman spectroscopy were carried out in an index case.Results:Out of the total 59 cases, 35 RB cases had different seedings. The mean age at enucleation was 2.9 years. RB seeds were seen in vitreous (n = 19), subretinal plus vitreous (n = 7), anterior chamber (n = 1), over crystalline lens (n = 3), retinal surface (n = 1), retinal pigment epithelium (RPE; n = 2), subretinal (n = 1), calcified seeds (n = 2). Other characteristics were dusts (n = 7), clouds (n = 11), spheres (n = 4), and unspecified type (n = 13). Histopathological high-risk factors showed significant choroidal (n = 22) and optic nerve (n = 15) involvement. Few cases had extraocular spread. Undifferentiated tumor (n = 24) was seen with higher evidence of necrosis (n = 23). Raman spectra differentiated the seeds from the normal tissue on the basis of lipid and protein content.Conclusion:This study highlights the different types of RB seeds with high-risk factors. The morphology of those seeds showed the difference between vitreous and subretinal seeds under advanced microscopic observations.

Intra-arterial Chemotherapy as Primary Treatment for Cavitary Retinoblastoma: Excellent Response in Eight Tumors

The "cavitary" form of retinoblastoma has historically demonstrated minimal treatment response with intravenous chemoreduction, showing less robust regression and less reduction in tumor size. Intra-arterial chemotherapy (IAC) has been reported to more effectively treat retinoblastoma, allowing many previously unsalvageable eyes to now be saved. The purpose was to report treatment response of cavitary retinoblastoma tumors to IAC. Retrospective case series. Patients presenting with cavitary retinoblastoma who were treated with IAC. Retrospective case series of all patients presenting with cavitary retinoblastoma between August 2014 and January 2019 who were treated with primary IAC. Tumor regression, recurrence, resolution of vitreous and subretinal seeds, number of treatments required, globe salvage, metastasis, and death. Eight cavitary retinoblastoma tumors in 6 eyes of 4 patients were treated with IAC. One hundred percent of the cavitary tumors regressed (8/8 tumors, in 6/6 eyes), and 100% of vitreous and subretinal seeds regressed, with 100% globe salvage. None of the tumors recurred, no patients developed metastases, and no patients died. Eyes were treated with a median of 4.5 cycles of IAC (range, 1-7), with fewer IAC treatments used in the later patients (1-3 treatments per eye for the most recent 3 eyes, compared with 6-7 treatments per eye for the earliest 3 eyes). Mean reduction in thickness was 73.4% (range, 59.7%-84.6%). Mean reduction in basal diameter was 45.5% (range, 24.8%-56.0%). Treatment with IAC results in regression of cavitary retinoblastoma, often with greater reduction in tumor size than has been reported previously with intravenous chemotherapy (IVC). Using up-front triple therapy (e.g., melphalan 0.4 mg/kg, carboplatin 50 mg, and topotecan 2 mg) and noting certain subtle signs of early regression can help to minimize unnecessary additional cycles of treatment.

Risk Factors for Tumor Recurrence Following Primary Intravenous Chemotherapy (Chemoreduction) for Retinoblastoma in 869 Eyes of 551 Patients.

To identify risk factors for retinoblastoma recurrence following chemoreduction. This was a retrospective review of patients with retinoblastoma treated from 1994 to 2019 using chemoreduction with analysis for recurrence using Kaplan-Meier, Cox regression, and logistic regression. There were 869 eyes of 551 patients with retinoblastoma treated with chemoreduction. Follow-up in 556 eyes revealed main solid tumor recurrence (n = 355, 64%), subretinal seed recurrence (n = 244, 44%), vitreous seed recurrence (n = 162, 29%), and/or new tumor (n = 118, 21%) requiring management with focal therapy (transpupillary thermotherapy, cryotherapy) (n = 294, 53%), intra-arterial chemotherapy (n = 125, 22%), intravitreal chemotherapy (n = 36, 6%), plaque radiotherapy (n = 120, 22%), external beam radiotherapy (n = 57, 10%), and/or enucleation (n = 49, 9%). Of all recurrences, 62% were detected by 1 year, 86% by 2 years, 94% by 3 years, 98% by 5 years, 99% by 10 years, and 100% by 15 years. Risk factors for recurrence on multivariate analysis included younger patient age at presentation (odds ratio [OR] = 1.02 [1.00 to 1.04] per 1 month decrease, P = .02), greater International Classification of Retinoblastoma group (OR = 1.24 [1.05 to 1.47] per 1 more advanced group, P = .01), shorter tumor distance to optic disc (OR = 1.11 [1.01 to 1.21] per 1 mm decrease, P = .03), and presence of subretinal seeds (OR = 1.66 [1.09 to 2.53], P = .02). Retinoblastoma recurrence after chemoreduction is usually detected within the first 3 years following treatment. Younger patients with more advanced, posteriorly located tumors and subretinal seeds at presentation are at increased risk, but recurrence can often be managed with globe-sparing therapy. [J Pediatr Ophthalmol Strabismus. 2020;57(4):224-234.].

Ophthalmic artery chemosurgery for retinoblastoma: an initial 3-year experience from two major institutes in Egypt

BackgroundIntra-arterial chemotherapy has shown promising results and improved the prognosis of retinoblastoma in the developed countries. Here, we aim to report our initial experience, in Egypt, in the treatment of all stages of naïve retinoblastoma with ophthalmic artery chemosurgery (OAC), using melphalan.ResultsThe technique was successful in 23 out of 26 eyes. Overall, a total of 63 treatment sessions were performed. Ophthalmic artery catheterization was performed in 59 treatment sessions, whereas middle meningeal artery catheterization was performed in 4 treatment sessions. Adequate tumor control was achieved in 95.6%. Progression has developed in one eye after the third OAC session. The mean follow-up period was 18.5 months. Recurrence of the main tumor as well as vitreous and subretinal seeds was reported in one eye after 19-month duration. A globe salvage rate of 91.3% was attained. No metastasis was recorded, and all patients were alive within the stated follow-up period.ConclusionA high globe salvage rate along with metastasis free survival and mild complications were achieved. Therefore, our initial experience with OAC in primary retinoblastoma proved to be safe and effective.

Long-term (20-year) real-world outcomes of intravenous chemotherapy (chemoreduction) for retinoblastoma in 964 eyes of 554 patients at a single centre

BackgroundIntravenous chemotherapy (IVC) remains an important globe salvage therapy for retinoblastoma.MethodsEvaluation of long-term globe salvage at 5, 10, 15 and 20 years following frontline IVC for retinoblastoma.ResultsOf 994 eyes, comparison...

Tumor Environment of Retinoblastoma, Intraocular Cancer.

Retinoblastoma, an intraocular cancer primarily affecting children, interacts with surrounding intraocular and extraocular structures in the development and progression. Subretinal and vitreous seeds are characteristic features of retinoblastoma, which result from the interaction between the tumor and its environment at the levels of tissue and microenvironment. The retina and vitreous affect the disease course and responses to treatment options. Also, neighboring cells in the retina and physicochemical properties of the tumor microenvironment are related to the biological activities of retinoblastoma tumors. Researches focusing on the tumor environment of retinoblastoma will lead to the development of more effective treatment options, which can revolutionize the prognosis of patients with retinoblastoma.

The Role of Intra-Arterial Chemotherapy in the Manegment of Retinoblastoma in Comparison with Other Modalities

AbstractBackground: Retinoblastoma often presents with advanced intraocular disease and despite conventional treatment with intravenous chemotherapy (IVC) and external beam radiation therapy, may still require enucleation.Aim of Study: To assess the efficacy of intra-arterial chemotherapy (IAC) in comparison with other modalities in the management of intraocular retinoblastoma.Material and Methods: Twenty one patients with thirty intra ocular retinoblastomas were included in the study from November 2013 to September 2016. Chemo reduction followed by adjuvant consolidative treatment has replaced external beam radiotherapy as the primary modality of treatment for intraocular retinoblastoma. Patients failed to respond ade-quately to a standard systemic chemotherapy (i.e., carboplatin, vincristine, and etoposide) with or without other local therapies, are referred for intra-arterial chemotherapy (IAC) to avoid enucleation. The intervention was selective catheterization of the ophthalmic artery and injection of chemotherapy.Results: The current commonly used treatments for retin-oblastoma in our study were IVC and IAC, along with con-solidated therapy based on tumor staging. For international classification of retinoblastoma (ICRB) grading, we found a significantly higher success rates for RTB cases treated with IAC as primary or adjuvant therapy compared to other treat-ment modalities which achieved 90% versus 73.6% for globe salvage, 80% vs 52.6 % for solid tumor control, 75% vs 66.6% for subretinal seeds control, 66.7% vs 62.5% for vitreous seeds control respectively.Conclusion: IAC may be superior to IVC for the treatment of retinoblastoma, with a higher overall success rate and higher globe salvage especially in group D retinoblastoma that failed to respond to systemic chemotherapy and destined for enucleation.