Ocular Surface Squamous Neoplasia with Coexistent Pterygia: A Study of 14 Cases and Review of Literature.

PURPOSE: To report the clinical presentation, anterior segment optical coherence tomography features, treatment, and outcomes of ocular surface squamous neoplasia (OSSN) associated with pterygium. METHODS: Retrospective interventional series of 14 cases in a 28-month study period. RESULTS: OSSN was coexistent with pterygium (n = 14) in < 1% of all pterygia (n = 7384). The mean age at the presentation of OSSN with pterygium was 49 years (median, 49 years; range, 36 to 71 years). Referral diagnosis included pterygium sans OSSN (n = 7, 50%), granuloma (n = 1, 7%), actinic keratosis (n = 1, 7%), and conjunctivitis (n = 1, 7%). All OSSNs were unilateral, and six patients (43%) had bilateral pterygia. Tumors arose from the nasal (n = 8, 57%), or temporal (n = 6, 43%) quadrants. The mean tumor diameter was 4 mm (median, 4 mm; range, 2 to 6 mm), and the mean thickness was 2 mm (median, 1 mm; range, 1 to 3 mm). The delineation between OSSN and pterygium could be identified on anterior segment optical coherence tomography (AS-OCT) in all (100%) cases. All patients received 1% topical 5-fluorouracil (5-FU), and complete tumor regression was achieved in 13 (93%) cases with a mean number of 2 cycles (median, two cycles; range, 1 to 4 cycles). There were no significant adverse effects. No tumor recurrence was noted over a mean follow-up period of 11 months (median 12 months; range, 1 to 4 months). CONCLUSION: AS-OCT allows accurate detection and mapping of tumor extent in OSSN with coexistent pterygium, and topical 5-FU yields excellent tumor control.

Differences in Childhood Growth Parameters Between Patients With Somatic and Heritable Retinoblastoma.

Purpose: Little is known regarding differences in childhood growth between somatic and heritable retinoblastoma (Rb) populations. We aimed to compare childhood growth parameters between somatic and heritable Rb cohorts at birth and at time of diagnosis with Rb. Methods: A multinational, longitudinal cohort study was conducted with patients from 11 centers in 10 countries who presented with treatment naïve Rb from January to December 2019. Variables of interest included age, sex, and size characteristics at birth and at time of presentation, as well as germline mutation status. After Bonferroni correction, results were statistically significant if the P value was less than 0.005. Results: We enrolled 696 patients, with 253 analyzed after exclusion criteria applied. Between somatic (n = 39) and heritable (n = 214) Rb cohorts, with males and females analyzed separately, there was no significant difference in birth weight percentile, weight percentile at time of diagnosis, length percentile at time of diagnosis, weight-for-length percentile at time of diagnosis, or change of weight percentile from birth to time of diagnosis. Patients with heritable Rb had a smaller mean weight percentile at birth and smaller mean weight and length percentiles at time of diagnosis with Rb, although this difference was not statistically significant. All cohorts experienced a slight negative change of weight percentile from birth to time of diagnosis. No cohort mean percentiles met criteria for failure to thrive, defined as less than the 5th percentile. Conclusions: Children with Rb seem to have normal birth and childhood growth patterns. There is no definitive evidence that somatic or heritable Rb has a biological or environmental impact on childhood growth parameters.

Small extracellular vesicles loaded with carboplatin effectively enhance the cytotoxicity of drug-resistant cells from Y79 cells-in vitro.

Drug resistance (DR) is one of the challenges in treating retinoblastoma (Rb) that warrants novel approaches. With the emerging evidence on the role of small extracellular vesicles (sEVs) as a drug-delivery carrier system, in this study, we derived the drug-resistant (DR) clones of Y79 cells and evaluated the efficacy of sEVs-loaded with carboplatin (sEVs-CPT) to reverse the chemoresistance. Drug-resistant clones of Y79 cells (DR-Y79) were systematically developed through sequential exposure to carboplatin (CPT), showcasing a sixfold increase in inhibitory concentration when compared to parental Y79 cells (IC50: 41.4 µg/mL and 6.2 µg/mL) (P<0.0001). These DR-Y79 cells show higher expression of ABCG2 and higher expression of DR genes than parental Y79 cells (P<0.0001). The sEVs were isolated from the conditioned media of Y79 cells using ultracentrifugation (UC) and characterized. Further, the sEVs were loaded with CPT and achieved higher encapsulation efficiency at one hour, and drug release of sEVs-CPT was highest at ∼80% at pH 5.0. The cytotoxicity of sEVs-CPT on Y79 cells and DR-Y79 was higher when compared to the CPT (IC50: 3.5 µg/mL vs 6.2 µg/mL; 23.1 µg/mL vs 41.2 µg/mL) (p<0.0001). This study demonstrates that sequential exposure to CPT generates DR clones of Y79 cells, which could serve as an appropriate model to evaluate the efficacy of drugs. The sEVs-CPT were highly effective in enhancing cytotoxicity in DR-Y79 cells, and appear to hold promise as a novel complimentary drug delivery system.

Indocyanine green-enhanced transpupillary thermotherapy for juxtapapillary retinal capillary hemangioblastoma.

PURPOSE: To study the clinical presentation and treatment outcomes of indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT) for treatment-naïve juxtapapillary retinal capillary hemangioblastoma (JRCH). METHODS: A prospective interventional case series. The technique involved ICG dye infusion 45 seconds prior to application of TTT. The main study outcomes were local tumor control, resolution of subretinal fluid (SRF), and improvement in best-corrected visual acuity (BCVA). RESULTS: Eight eyes of seven patients (5 males and 2 females) were included. The mean age was 26 years (range: 5-56 years). Systemic evaluation revealed von-Hippel Lindau (VHL) disease in five patients. The most common location was the temporal aspect of the optic disc (5 eyes). The mean basal diameter was 2.9 mm (range: 1-8 mm), and tumor thickness was 1.4 mm (range: 1-4 mm). All eight eyes were treated with multiple sessions of ICG-TTT (mean: 3 sessions). Six eyes received adjuvant intravitreal injection of dexamethasone implant (4 eyes) and/or bevacizumab (4 eyes). Post treatment, six eyes (75%) had tumor regression with reduction of SRF. One eye had a partial response with persisting SRF, and one eye showed poor response to TTT for which external beam radiotherapy was performed. At the last follow-up (median: 11 months; range: 6-29 months), the BCVA remained stable in seven eyes and improved in one eye (hand motion to 20/40). CONCLUSION: Multiple ICG-TTT sessions can be considered as an alternative treatment option for JRCH with effective local tumor control and SRF resolution.

Generation of an induced pluripotent stem cell line (LVPEIi002-A) with heterozygous RB1 mutation using peri-orbital fat derived mesenchymal cells of a patient with inherited retinoblastoma.

Retinoblastoma is a pediatric intraocular cancer caused by biallelic inactivation of RB1 gene in retinal progenitor cells. Here, we report the generation of a patient-specific induced pluripotent stem cell (iPSC) line (LVPEIi002-A) from a patient diagnosed with retinoblastoma and showing familial inheritance of a nonsense mutation (c.1735C > T) within exon 18 of one of the two alleles. This RB1+/- iPSC line, LVPEIi002-A was generated by reprogramming the peri-orbital fat tissue derived mesenchymal cells and was stably expanded and characterized. It maintains the stemness, pluripotency, normal karyotype, and forms embryoid bodies comprising of all three lineage committed progenitor cells.

Global retinoblastoma studies: A review.

In the current era of global health awareness for retinoblastoma (RB), the challenge that lies ahead of us is providing optimal care for children affected with RB in underdeveloped nations. The understanding of similarities and disparities between various nations across the world aids in achieving comparable outcomes. With dissolving geographic barriers and evolving collaboration, global collaborative studies on RB are becoming increasingly common. They provide real-world, robust evidence on several aspects of RB. This review discusses insights gained from global RB studies regarding the demographics, certain aspects of etiopathogenesis and epidemiology, international travel burden, disparities in clinical presentations based on national income levels, management protocols, pathology, treatment outcomes, and the effect of COVID-19 on RB care across the world. These insights are likely to impact individual practice as well as inform policy reforms.

Retinoblastoma in Asia: Clinical Presentation and Treatment Outcomes in 2112 Patients from 33 Countries.

PURPOSE: To describe the clinical presentation and treatment outcomes of children who received a diagnosis of retinoblastoma in 2017 throughout Asia. DESIGN: Multinational, prospective study including treatment-naïve patients in Asia who received a diagnosis of retinoblastoma in 2017 and were followed up thereafter. PARTICIPANTS: A total of 2112 patients (2797 eyes) from 96 retinoblastoma treatment centers in 33 Asian countries. INTERVENTIONS: Chemotherapy, radiotherapy, enucleation, and orbital exenteration. MAIN OUTCOME MEASURES: Enucleation and death. RESULTS: Within the cohort, 1021 patients (48%) were from South Asia (SA), 503 patients (24%) were from East Asia (EA), 310 patients (15%) were from Southeast Asia (SEA), 218 patients (10%) were from West Asia (WA), and 60 patients (3%) were from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, < 1-261 months). The cohort included 1195 male patients (57%) and 917 female patients (43%). The most common presenting symptoms were leukocoria (72%) and strabismus (13%). Using the American Joint Committee on Cancer Staging Manual, Eighth Edition, classification, tumors were staged as cT1 (n = 441 [16%]), cT2 (n = 951 [34%]), cT3 (n = 1136 [41%]), cT4 (n = 267 [10%]), N1 (n = 48 [2%]), and M1 (n = 129 [6%]) at presentation. Retinoblastoma was treated with intravenous chemotherapy in 1450 eyes (52%) and 857 eyes (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, and 20% and 11% for WA (P < 0.0001 and P < 0.0001), respectively. CONCLUSIONS: At the conclusion of this study, significant heterogeneity was found in treatment outcomes of retinoblastoma among the regions of Asia. East Asia displayed better outcomes with higher rates of globe and life salvage, whereas Southeast Asia showed poorer outcomes compared with the rest of Asia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

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

PURPOSE: To determine the efficacy of secondary salvage intravenous chemotherapy (IVC) for refractory/recurrent retinoblastoma. METHODS: Retrospective, nonrandomized interventional case series of 41 eyes of 33 patients with recurrent retinoblastoma. RESULTS: 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. CONCLUSION: 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.

Ocular surface pseudoepitheliomatous hyperplasia secondary to allergic eye disease: clinical features and management.

PURPOSE: To study the clinical characteristics and treatment outcomes of ocular surface pseudoepitheliomatous hyperplasia (PEH) associated with chronic vernal keratoconjunctivitis (VKC). METHODS: This retrospective study includes 39 eyes of 32 patients with VKC induced PEH who presented from 2016-2022. A database search was conducted for diagnosis of PEH, and data on clinical features, imaging characteristics, and treatment were analyzed. RESULTS: Of the 32 patients, 11 (34%) were children and adolescents, 21 (66%) were adults. PEH was common in males (72%) and ocular surface squamous neoplasia (OSSN) was the commonest referral diagnosis (43.7%). Mean age at presentation was 26.62 ± 10.18 (range: 6-52) years. While history of VKC was present in 21 patients, 11 were diagnosed with VKC at the time of diagnosis of PEH. The mean base/largest diameter was 5.2 ± 1.67 mm. Anterior segment optical coherence tomography (AS-OCT) showed irregular hyper-reflective epithelium, epithelial dipping, and sub-epithelial hyper-reflective lesion with shadowing in all lesions. Of the 31 eyes that received medical therapy, 21 (67%) and 10 (32%) eyes showed complete and partial resolution respectively with median time to resolution of 3(IQR:2-4) months. Eight eyes that underwent surgical excision showed complete resolution and one developed partial limbal stem cell deficiency. CONCLUSION: Ocular surface PEH is a manifestation of chronic VKC which closely mimics OSSN. Detailed history-taking, examination for signs of allergy, and AS-OCT imaging can distinguish it from OSSN. It responds well to medical therapy and should be considered first-line therapy before planning any surgical intervention.

Metastatic retinoblastoma at presentation: Clinical presentation, treatment, and outcomes.

The aim of this study was to retrospectively determine clinical features, treatment outcomes, and overall survival in four patients with metastatic retinoblastoma at presentation. The mean age at diagnosis was 63 months (range: 24-108 months). Three patients had overt orbital disease of at least one eye and one patient had microscopic orbital disease with scleral infiltration on histopathology. Metastatic sites included regional lymph nodes (RLN) (n = 4), bone marrow (BM) (n = 2), and cerebrospinal fluid (CSF) (n = 1). The most common sites of RLN were ipsilateral preauricular nodes (two patients) and contralateral parotid gland involvement (one patient). The treatment administered included primary enucleation (n = 1), high-dose intravenous chemotherapy (n = 4), secondary enucleation (n = 2), orbital external beam radiotherapy (n = 3), and intrathecal chemotherapy (n = 1). High-risk features included massive choroidal and microscopic scleral infiltration in the eye that underwent primary enucleation. At a mean follow-up of 33 months (range, 4-68 months), one patient with CSF involvement deceased in 4 months. The remaining three patients were alive and disease-free at the last mean follow-up period of 43 months (range, 18-68 months). The results of our study showed that RLN and BM metastasis respond well to treatment while CSF metastasis is associated with poor prognosis.

Serum Metabolomics of Retinoblastoma: Assessing the Differential Serum Metabolic Signatures of Unilateral and Bilateral Patients.

Retinoblastoma (Rb) is the most common pediatric eye cancer. To identify the biomarkers for early diagnosis and monitoring the progression of Rb in patients, mapping of the alterations in their metabolic profiles is essential. The present study aims at exploring the metabolic disparity in serum from Rb patients and controls using NMR-based metabolomics. A total of 72 metabolites, including carbohydrates, amino acids, and organic acids, were quantified in serum samples from 24 Rb patients and 26 controls. Distinct clusters of Rb patients and controls were obtained using the partial least-squares discriminant analysis (PLS-DA) model. Further, univariate and multivariate analyses of unilateral and bilateral Rb patients with respect to their age-matched controls depicted their distinct metabolic fingerprints. Metabolites including 2-phosphoglycerate, 4-aminobutyrate, proline, O-phosphocholine, O-phosphoethanolamine, and Sn-glycero-3-phosphocholine (Sn-GPC) showed significant perturbation in both unilateral and bilateral Rb patients. However, metabolic differences among the bilateral Rb cases were more pronounced than those in unilateral Rb cases with respect to controls. In addition to major discriminatory metabolites for Rb, unilateral and bilateral Rb cases showed specific metabolic changes, which might be the result of their differential genetic/somatic mutational backgrounds. This further suggests that the aberrant metabolic perturbation in bilateral patients signifies the severity of the disease in Rb patients. The present study demonstrated that identified serum metabolites have potential to serve as a noninvasive method for detection of Rb, discriminate bilateral from unilateral Rb patients, and aid in better understanding of the RB tumor biology.

Pediatric ocular melanoma: a collaborative multicenter study and meta-analysis.

PURPOSE: To investigate clinical manifestations and prognoses in pediatric patients (≤12 years old) with ocular melanoma. METHODS: This was a retrospective, multicenter cohort study with individual participant data (IPD) meta-analysis pooling available published cases, and unpublished cases from an international collaboration of seven ocular oncology centers. RESULTS: There were 133 eyes of 133 pediatric patients with choroidal or ciliary body (n = 66 [50%]), iris (n = 33 [25%]), conjunctival (n = 26 [19%]), and eyelid (n = 8 [6%]) melanoma. Overall, the mean patient age at presentation was 7 years (median, 8; range, 1-12 years), with 63 males (49%). The mean age by tumor site was 6.50 ± 3.90, 7.44 ± 3.57, 9.12 ± 2.61, and 5.63 ± 2.38 years, for choroid/ciliary body, iris, conjunctiva, and eyelid melanoma, respectively (P = 0.001). Association with ocular melanocytosis was seen in 15%, 11%, 4%, and 0%, respectively (P = 0.01). Frequency of ocular melanoma family history did not vary by tumor site (7%, 17%, 9% and 12%, resp. [P = 0.26]). After mean follow-up of 74, 85, 50, and 105 months (P = 0.65), metastasis was seen in 12%, 9%, 19%, and 13% of choroid/ciliary body, iris, conjunctiva, and eyelid melanoma, respectively. Death was reported in 5%, 3%, 8%, and 0%, respectively, with survival analysis indicating higher mortality in choroidal/ciliary body and conjunctival melanoma patients. CONCLUSIONS: Ocular melanoma in the pediatric population is rare, with unique clinical features and outcomes. Iris melanoma accounts for about one-third of pediatric uveal melanoma cases.

Identifying Treatment Resistance Related Pathways by Analyzing Serum Extracellular Vesicles of Patients With Resistant Versus Regressed Retinoblastoma.

Purpose: To identify the genes and pathways responsible for treatment resistance (TR) in retinoblastoma (RB) by analyzing serum small extracellular vesicles (sEVs) of patients with TR active RB (TR-RB) and completely regressed RB (CR-RB). Methods: Serum-derived sEVs were characterized by transmission electron microscopy and nanoparticle tracking analysis. sEV transcriptome profiles of two TR-RB and one CR-RB with good response (>20 years tumor free) were compared to their age-matched controls (n = 3). Gene expression data were analyzed by the R Bioconductor package. The CD9 protein and mRNA expression of CD9, CD63, and CD81 were studied in five RB tumors and two control retinae by immunohistochemistry and quantitative reverse transcription-polymerase chain reaction. Results: The isolated serum sEVs were round shaped and within the expected size (30-150 nm), and they had zeta potentials ranging from -10.8 to 15.9 mV. The mean ± SD concentrations of sEVs for two adults and four children were 1.1 × 1012 ± 0.1 and 5.8 × 1011 ± 1.7 particles/mL. Based on log2 fold change of ±2 and P < 0.05 criteria, there were 492 dysregulated genes in TR-RB and 184 in CR-RB. KAT2B, VWA1, CX3CL1, MLYCD, NR2F2, USP46-AS1, miR6724-4, and LINC01257 genes were specifically dysregulated in TR-RB. Negative regulation of apoptotic signaling, cell growth, and proton transport genes were greater than fivefold expressed only in TR-RB. CD9, CD63, and CD81 mRNA levels were high in RB tumors versus control retina, with increased and variable CD9 immunoreactivity in the invasive areas of the tumor. Conclusions: Serum sEVs could serve as a potential liquid biopsy source for understanding TR mechanisms in RB.

Anterior segment optical coherence tomography features of ocular surface squamous epithelial hyperplasia: A report of four cases.

Herein, we report the clinico-tomographic and histopathological features of four patients with biopsy-proven ocular surface squamous epithelial hyperplasia (OSSEH), a close mimicker of ocular surface squamous neoplasia (OSSN). The mean age at diagnosis was 58 years (median, 60 years; range, 35-77 years). All lesions were unilateral. Isolated corneal plaque was seen in 50% (n = 2) and nodular lesion at the nasal limbus in 50% (n = 2). Keratinization was seen in 75% (n = 3) of lesions and intrinsic vessels in 75% (n = 3). A clinical diagnosis of OSSN was made in all cases. Anterior segment optical coherence tomography (ASOCT) revealed mild epithelial hyperreflectivity in 100% (n = 4). The epithelium was normal in thickness in 75% (n = 3) and showed mild thickening in 25% (n = 1). Only 25% (n = 1) showed abrupt transition in epithelial thickness from the contiguous corneal epithelium. Histopathological examination revealed hyperplastic squamous epithelium and no cellular atypia in all cases. Stable ocular surface and no recurrences were noted at a mean follow-up of 17 months (median, 11 months; range, 2-43 months). Although OSSEH can mimic OSSN clinically, the presence of mild epithelial hyperreflectivity, lack of epithelial thickening, absence of abrupt transition from normal epithelium, and presence of subepithelial hyperreflectivity on ASOCT favor the diagnosis of OSSEH.

Corneal squamous neoplasia: masquerades and management outcomes at a rural eyecare centre.

The authors describe two cases of corneal ocular surface squamous neoplasia (OSSN), presenting at our rural eyecare centre, which were initially misdiagnosed as viral epithelial keratitis and corneal pannus with focal limbal stem cell deficiency. Both the cases were refractory to initial treatment and corneal OSSN was suspected. Anterior segment-optical coherence tomography (AS-OCT) revealed a thickened, hyper-reflective epithelium with abrupt transition and an underlying cleavage plane, features typical of OSSN. Topical 1% 5-fluorouracil (5-FU) therapy was initiated and in two cycles (first case) to three cycles (second case), complete resolution was noted both clinically and on AS-OCT, with no significant side effects. Both patients are currently free of tumour at the 2-month follow-up period. The authors report the rare, atypical presentations of corneal OSSN, discuss the masquerades and highlight the role of primary topical 5-FU in managing corneal OSSN in limited resource settings.

Artificial intelligence and machine learning in ocular oncology: Retinoblastoma.

Purpose: This study was done to explore the utility of artificial intelligence (AI) and machine learning in the diagnosis and grouping of intraocular retinoblastoma (iRB). Methods: It was a retrospective observational study using AI and Machine learning, Computer Vision (OpenCV). Results: Of 771 fundus images of 109 eyes, 181 images had no tumor and 590 images displayed iRB based on review by two independent ocular oncologists (with an interobserver variability of <1%). The sensitivity, specificity, positive predictive value, and negative predictive value of the trained AI model were 85%, 99%, 99.6%, and 67%, respectively. Of 109 eyes, the sensitivity, specificity, positive predictive value, and negative predictive value for detection of RB by AI model were 96%, 94%, 97%, and 91%, respectively. Of these, the eyes were normal (n = 31) or belonged to groupA (n=1), B (n=22), C (n=8), D (n=23),and E (n=24) RB based on review by two independent ocular oncologists (with an interobserver variability of 0%). The sensitivity, specificity, positive predictive value, and negative predictive value of the trained AI model were 100%, 100%, 100%, and 100% for group A; 82%, 20 21 98%, 90%, and 96% for group B; 63%, 99%, 83%, and 97% for group C; 78%, 98%, 90%, and 94% for group D, and 92%, 91%, 73%, and 98% for group E, respectively. Conclusion: Based on our study, we conclude that the AI model for iRB is highly sensitive in the detection of RB with high specificity for the classification of iRB.

RETINOBLASTOMA ASSOCIATED WITH TOTAL EXUDATIVE RETINAL DETACHMENT: Treatment and Outcomes.

PURPOSE: To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma and total retinal detachment. METHODS: A retrospective study of 50 eyes in 43 patients. RESULTS: The mean age at presentation was 15 months (median, 9 months). Retinoblastoma-associated total retinal detachment 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 (n = 4; 8%). Based on eighth 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 intraarterial chemotherapy (n = 2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7-62 months), retinal detachment resolved in 41 eyes (82%), tumor recurrence was noted in 39 eyes (78%), globe salvage was achieved in 35 eyes (70%), and one patient (2%) died due to metastasis. CONCLUSION: Relatively high tumor recurrence rate is noted in retinoblastoma presenting with total retinal detachment. With appropriate treatment, 70% of the globes can be salvaged.

Ocular surface squamous neoplasia with 360° limbal involvement: a study of 130 patients.

PURPOSE: To describe the risk factors, clinical features and management outcomes of ocular surface squamous neoplasia (OSSN) with 360° of limbal involvement (360-OSSN) and compare with segmental limbal involvement (SL-OSSN). METHODS: Retrospective comparative study of 360-OSSN vs SL-OSSN. All 360-OSSN and every 10th patient with SL-OSSN during the study period (2012-2020) were included. Lesions with uncertain diagnosis were excluded. RESULTS: Of 1250 patients diagnosed with OSSN during the study period, 30 (2%) had 360-OSSN. A total of 100 patients of OSSN with SL-OSSN were included for comparison. 360-OSSN patients more often had longer duration of symptoms (mean, 17 vs 8 months; p, 0.003), prior misdiagnosis (17% vs 6%, p, 0.13) and prior intervention (47% vs 13%; p, 0.0002) than patients with SL-OSSN. 360-OSSN had higher incidence of scleral fixity (57% vs 16%; p < 0.0001), corneal/scleral melt (17% vs 0%; p, 0.0005), intraocular tumor extension (17% vs 0%; p, 0.003), orbital tumor extension (33% vs 1%; p < 0.0001), and advanced T stage at presentation (Tis: 37% vs 76%, T1: 0% vs 15%; T2: 7% vs 4%; T3: 27% vs 4%; T4: 30% vs 1%; p < 0.001). Over a mean follow-up of 14 months, lymph node metastasis (8% vs 0%; p, 0.05) and distant metastasis (4% vs 0%; p, 0.23) were more common in 360-OSSN group compared to SL-OSSN group. CONCLUSION: Risk factors of 360-OSSN include prolonged symptoms, prior misdiagnosis and prior intervention. It represents an advanced form of disease with propensity for corneo-scleral melt and invasive disease which requires aggressive management.

Vasoproliferative retinal tumors: Clinical presentation and treatment outcome.

OBJECTIVE: To study the clinical presentation and treatment outcomes of patients with vasoproliferative retinal tumor (VPRT) in India. METHODS: Retrospective study of 50 eyes in 47 patients. RESULTS: Of the 50 eyes, 25 (50%) were primary and 25 (50%) were secondary. Common ocular pathologies related to secondary VPRT included retinal detachment (n = 8, 32%) and Coats disease (n = 7, 28%). Overall, the mean age at presentation was 35 years (range; 3-74 years) and included 30 (64%) males and 17 (36%) females. The lesions were unilateral in 44 (94%) patients. Secondary retinal features included intra/subretinal exudation (n = 41, 82%), vascularity (n = 32, 64%), subretinal fluid (n = 21, 42%), retinal neovascularisation (n = 9, 18%) and vitreous hemorrhage (n = 8, 16%). Thirty-four eyes (68%) underwent cryotherapy, of which 16 eyes (47%) received adjunct intravitreal anti-VEGF (12 eyes) or corticosteroid injection (4 eyes). Primary surgery included vitrectomy with/without encirclage and cryotherapy (n = 12, 24%) and plaque brachytherapy (n = 3, 6%). At last follow-up (mean 9.7 months, range 3-67 months), 42 eyes (84%) had complete tumor regression with no improvement in visual outcomes when comparing presenting and final best corrected visual acuity. Comparing primary versus secondary VPRT, secondary VPRT presented a decade earlier (31 vs 40 years), needed surgical intervention (48% vs 20%), had poor tumor control (72% vs 92%) and worse visual outcomes (p < 0.05). CONCLUSION: VPRTs commonly present as a unilateral lesions in the adult population with equal prevalence of primary and secondary variants. Compared with primary VPRT, secondary VPRTs require surgical treatment and are associated with poor visual outcome.