Background: Extensive literature exists on characterizing and differentiating eyelid neoplasms that may mimic benign lesions. However, there is limited epidemiologic data on the most common benign and malignant neoplasms of the eyelid, especially within the United States. Methods: A retrospective cohort study was performed of all histopathologically-confirmed eyelid biopsy specimens between 1/2000 - 12/2021 at a large tertiary care academic medical center. Age, sex, lesion site and laterality, and diagnosis was collected. Specimens were stratified into benign and malignant eyelid lesions. Results: 561 eyelid cases (male = 270, female = 291) were analyzed, 460 benign (82.0%) and 101 malignant (18.0%). Mean age for benign lesions was 60.6 ± 15.2 years. Mean age for malignant lesions was 67.6 ± 12.5 years. Most common benign lesions were papillomas (n=146: 31.7%), hidrocystoma (n=65: 14.1%), and epidermal inclusion cyst (n=41: 8.9%). Most common malignant lesions were basal cell carcinoma (n=72: 71.3%), squamous cell carcinoma (n=17: 16.8%), and melanoma (n=5: 5.0%). Conclusions: The number of benign eyelid lesions outweighs those of malignant lesions. Benign lesions were commonly seen at an earlier age compared to malignant lesions. Epidemiologic data presented adds to knowledge about lesions and can help inform clinical differential diagnoses.
Introduction:The aim of this study was to characterize optical coherence tomography (OCT) findings in choroidal osteoma and evaluate their associations with subretinal fluid, choroidal neovascularization, and structural retinal changes. Methods:This retrospective descriptive case series included 7 patients (10 eyes) with choroidal osteoma evaluated at a tertiary referral center. All eyes underwent comprehensive ophthalmologic examination and spectral-domain OCT imaging. OCT scans were analyzed for predefined structural features, including hyperreflective lamellar patterns, spongiform appearance, outer retinal alterations, and subretinal fluid. Subretinal fluid was classified as neovascular or non-neovascular based on OCT characteristics. Results:OCT revealed marked structural heterogeneity. Three eyes demonstrated isolated subretinal fluid without neovascular features, resolving spontaneously in two cases. Three eyes showed chronic subretinal fluid or intraretinal cystic changes with neovascular OCT features and responded favorably to repeated intravitreal anti-vascular endothelial growth factor therapy. One eye with long-term follow-up exhibited progressive tumor decalcification with severe retinal and choroidal thinning and poor visual outcome. Focal choroidal excavation was identified in one eye in association with choroidal neovascularization. Conclusion:Choroidal osteoma exhibits heterogeneous OCT patterns reflecting different mechanisms of visual impairment. Careful OCT-based assessment may aid differentiation of neovascular and non-neovascular changes and guide clinical management.
Introduction: Mirvetuximab soravtansine is an antibody-drug conjugate approved for the treatment of folate receptor alpha-expressing ovarian cancer and is associated with ocular adverse events. Patients treated with mirvetuximab soravtansine often develop moderate to severe keratopathy, requiring topical steroids. Recent studies suggest a higher incidence of cataract formation than initially reported. This case series describes the rapid development of visually significant cataracts in 3 patients receiving mirvetuximab therapy. Case Presentations: Three female patients undergoing treatment with mirvetuximab soravtansine for platinum-resistant serous ovarian cancer experienced significant visual decline after 11, 18, and 19 treatment cycles, in 1 case progressing from 20/25 to counting fingers in both eyes within 10 weeks. All patients had baseline ophthalmic examinations and were treated with prophylactic topical corticosteroids to mitigate corneal complications. Despite standard management, each patient developed bilateral posterior subcapsular cataracts with substantial loss of visual acuity. Patients underwent sequential or same-day bilateral cataract extraction without complication. Postoperatively, all patients regained their baseline vision, and oncologic therapy was continued. Conclusion: Rapidly progressing posterior subcapsular cataracts may occur in patients receiving prolonged mirvetuximab soravtansine therapy and may reflect drug-related toxicity, steroid exposure, or a synergistic interaction. Given that these patients did not develop cataracts until later cycles, ophthalmologic monitoring may be warranted beyond the duration specified in current treatment protocols. Early recognition and cataract surgery can restore vision without requiring cessation of oncologic therapy.
Introduction:Uveal melanoma (UM) is a challenging malignancy, in terms of diagnosis, risk stratification, and treatment associated with high morbidity and mortality rates. It has been demonstrated that E2F-related pathways play a significant role in the tumorigenesis and distant metastasis of UM. In this study, the E2F target-related genes were utilized to construct and validate a prognostic risk score for patients with UM. Methods:Using the TCGA-UVM cohort (n = 80), 192 E2F target genes were screened using gene set enrichment analysis (GSEA) to identify survival-associated genes. Prognostic genes were filtered using Kaplan-Meier analysis, univariate Cox regression, and LASSO, followed by multivariate Cox regression to construct a risk score model. The model was validated using the GSE22138 cohort (n = 63). Functional annotations of the risk score and its impact on stratifying tumor immune microenvironment components were assessed. Results:A total of 9 genes (CDC25B, NME1, RFC2, PRDX4, NASP, UBE2S, PRKDC, MCM6, and LBR) passed the model construction pipeline. The risk score categorization system showed an independent prognostic power (HR: 2.34, 95% CI: 1.11-4.90, p = 0.025) and a good predictability of the survival outcome (receiver operating characteristic curve analysis: area under the curve = 0.730, 95% CI: 0.60-0.86, p = 0.002). When analyzing the most frequently mutated gene cohorts, the risk score was significantly lower in the mutated subgroups of GNAQ, SF3B1, and EIF1AX. In contrast, the risk score was notably higher in the BAP1 mutated subgroup. Copy number analysis of chromosomal arms showed significant correlations between the risk score and 1q, 3q, 3p, 6p, 8q. The high-risk group showed significant infiltration for NK cells, plasma B cells, gamma delta T cells, follicular T cells, M1 and M2 macrophages with lower infiltration of common myeloid progenitor cells. In addition, the high-risk group showed higher immune and microenvironment scores. Conclusion:The developed E2F target-related gene model offers a robust tool for predicting the prognosis of UM patients. As a potential risk stratification method for UM, this model could have clinical applications pending further evaluation.
Introduction: Diffusion-weighted (DWI) and perfusion-weighted MRI (PWI) can assess biological tumour characteristics of uveal melanoma (UM), the most common primary intraocular malignant tumour in adults. Recent studies propose that these techniques can aid in the differential diagnosis, provide prognostic information, and enable early assessment of treatment response following radiation therapy. However, it is important to determine the extent to which any observed differences can be attributable to true biological changes in the tumour rather than day-to-day physiological or measurement variations. Therefore, this study aims to assess the reproducibility of ocular DWI and PWI measurements between two centres. Method: In this prospective study, 15 patients with UM were included. Each patient underwent two MRIs at two different centres with a mean interval of 28 days (range: 12-43 days). Apparent diffusion coefficient (ADC), relative peak intensity and outflow percentage of the tumour were compared between the two timepoints. Mean absolute differences between the two timepoints were calculated. Results: Mean absolute differences between the two timepoints were 0.07 × 10-3 mm²/s (95% CI, 0.04-0.11) for ADC, 0.11 (95% CI, 0.04-0.18) for relative peak intensity and 6 (95% CI, 3-10) percentage points for outflow percentage. Conclusion: This study shows that DWI and PWI parameters demonstrate reproducibility in UM patients between two different centres, with variabilities smaller than differences typically observed in differential diagnosis or treatment response assessments.
Introduction:This single-institution study characterizes outcomes in periocular sebaceous carcinoma and evaluates whether treatment delay is associated with orbital exenteration. Methods:Retrospective case series of all patients diagnosed with primary periocular sebaceous carcinoma between January 1, 2000, and December 31, 2022, at a single academic institution. Clinical, histopathologic, treatment, and outcome data were analyzed. Association between time to treatment and orbital exenteration was assessed using logistic regression. Results:The study included 25 patients (median age 76.6 years). Prior misdiagnosis occurred in 21 (84%), most commonly as chalazia (n = 7) or blepharitis (n = 6). Most tumors were AJCC T1, with predominantly poor or moderate differentiation. Mean time from presentation-to-treatment was 5.67 months (range 0.26-28.52 months), with a subset experiencing prolonged delays. Most patients underwent wide local excision (52%) or Mohs surgery (32%), with 4 (16%) requiring exenteration. Each 3-month increase in time to treatment was associated with 16% greater odds of orbital exenteration (OR: 1.16, 95% CI: 1.01-1.38; p = 0.042). Demographic and socioeconomic variables were not associated with differences in delays or outcomes. Median follow-up was 43 months, during which visual acuity remained stable in non-exenterated patients (median logMAR change -0.125). Local tumor recurrence occurred in 4 (16%) at a median of 26.46 months. Overall survival was 54.2% at 10 years, with no deaths attributed to sebaceous carcinoma. Conclusion:Treatment delay was associated with increased odds of orbital exenteration and not with measured socioeconomic factors. These findings suggest a potential relationship between time-to-treatment and extent of surgical management, with possible implications for vision preservation and quality of life.
Introduction:Evidence-based medicine relies on publicly available research findings, and the non-publication of studies may predispose to incomplete evidence on intervention efficacy or redundant funding for re-attempting unpublished studies. This study investigated non-publication rates among US-registered interventional studies for uveal melanoma. Methods:In April 2025, ClinicalTrials.gov was searched for interventional uveal melanoma studies. Interventional studies were included if completed at least 3 years prior to the database search, excluding completed trials awaiting results publication. Studies were labeled as having publicly available results if data were posted through ClinicalTrials.gov or in a journal publication. For published studies, we determined whether their primary study endpoint result was statistically significant. For all records, we collected study characteristics, including phase, sponsor, country of study, year of study initiation, enrollment, melanoma type, treatment type, and randomization status. The primary outcome was the overall rate of non-publication among all records. Second, we provided descriptive characteristics of included records and compared rates of non-publication between sublevels of primary endpoint significance status and study characteristics. Wherever applicable, the Fisher-exact, Wilcox rank-sum, and binomial exact tests analyzed these comparisons. Analysis was completed in RStudio (v2022.02 + 433). Results:From 102 records, we identified 98 trials (N = 4,669) for final inclusion. Overall, 38% of records did not have an identifiable online publication on any platform. Whether published or unpublished, 23% and 16% of trials were terminated, respectively, and the reasons for termination were heterogeneous. Trials with industry sponsorship were significantly more often published than those without industry sponsorship (p = 0.024). No significant association was found between publication status and study phase, enrollment size, study duration, or randomization status. Among the 60 studies published online, 64% had reported significant primary study endpoints. Given this, the calculated probability of publication to ClinicalTrials.gov or in a journal was 65% [95% CI: 51.60-76.87%; p = 0.03]. No significant association was found between significant positive primary outcome results and randomization status or sponsor type. Conclusion:About one-third of US-registered uveal melanoma interventional studies were not published online. Greater transparency of results or reasons for non-publication is needed.
Introduction:Chronic macular edema is a well-recognized complication of radiation-induced maculopathy (RM). Current treatment methods vary in efficacy and clinical benefit. The potential of fluocinolone acetonide (FAc) slow-release intravitreal implants (Iluvien® implant) remains unclear. We hypothesized that local continuous delivery of low-dose corticosteroids may improve RM symptoms. Methods:This study included 8 patients who presented with RM after 106-Ru-plaque brachytherapy (n = 3), proton beam therapy (n = 2), or stereotactic radiation therapy (n = 3) with photons. All eyes were initially treated with triamcinolone injections but proved refractory to steroids. The Iluvien® implant was injected, and patients were followed to monitor clinical improvement. Results:After 5 years, visual acuity improved in 3 patients, remained stable in 2 patients, and decreased in 2 patients. One patient progressed to no light perception following treatment. One patient was lost to follow-up after 5 years. Central macular thickness was reduced in 5 patients, stable in 1 patient, and increased in 1 patient. Discussion:Slow-release FAc implants are a promising treatment option to improve the anatomical structure of the fovea and visual function. They may also reduce the frequency of intravitreal injections and the overall therapeutic burden.
Introduction:Uveal melanoma (UM) is a rare malignancy in Latin America (LA), where coordinated clinical and translational research initiatives remain limited. We evaluated the implementation of a national referral and biobanking program for UM in Chile and assessed the feasibility of liquid biopsy workflows in a middle-income healthcare setting. Methods:We established a coordinated national clinical and translational research program for UM in Chile, integrating centralized referral, standardized workflows, biobanking, and longitudinal sampling. Patients were prospectively recruited through a national referral network between 2021 and 2025. Both solid tissue and liquid biopsy biospecimens were processed under standardized protocols and assessed for downstream molecular analyses. Results:A total of 45 patients with UM were recruited nationwide. A total of 76 biospecimen sets were processed with complete pre-analytical traceability. Profiling of plasma-derived cell-free DNA showed characteristic fragment patterns. Formalin-fixed paraffin-embedded (FFPE)-derived tumor DNA and paired non-tumor (matched) DNA met quality thresholds for next-generation sequencing (NGS). However, in this pilot setting, circulating tumor DNA (ctDNA) quantities were insufficient to consistently recover the full spectrum of tumor-associated genomic alterations identified in matched FFPE samples through NGS. Conclusions:This study demonstrates the feasibility of implementing a coordinated national clinical and translational research framework for UM within a middle-income healthcare setting with heterogeneous access to specialized molecular infrastructure. While standardized biospecimen collection and liquid biopsy workflows can be successfully established, limitations related to ctDNA abundance and persistent disparities in geographic and financial access to specialized care remain. This national model provides a scalable foundation for future multicenter studies, prospective biomarker validation, and biomarker-driven and molecularly informed studies in LA UM populations.
Background: Ocular metastases include both intraocular and ocular adnexal metastases. Although rare, they are important indicators of systemic malignancy and can even be the first sign of cancer. Breast and lung carcinomas account for the maximum number of cases; kidney, gastrointestinal tract, and cutaneous melanomas are less frequent primaries. The choroid is the most common site of metastasis because of its rich vascular flow. Literature on ocular metastases remains limited, necessitating a comprehensive understanding of their clinical behavior, diagnostic approach, and management. Summary: This review provides a comprehensive overview of the epidemiology, clinical features, and diagnosis of these lesions. Tumor spread primarily occurs via the hematogenous route, with organ-specific predilection explained by vascular and microenvironmental factors. Choroidal metastases are the most common intraocular presentation, while orbital involvement predominates among adnexal sites. Clinical manifestations vary widely; advances in ophthalmic multimodal imaging and intraocular biopsy techniques facilitate early detection of ocular metastases. Although systemic chemotherapy and radiotherapy remain the cornerstones of treatment, the advent of newer therapeutic agents like immunotherapy and targeted therapy has revolutionized the management of these malignancies. Local treatment options like plaque brachytherapy, local resection, and photodynamic therapy are used in selective cases. Emerging techniques, including liquid biopsy and targeted molecular therapies, are expanding treatment possibilities. Key Messages: Ocular metastases serve as important indicators of systemic cancer; they may precede the diagnosis of the primary malignancy, requiring prompt recognition. A high index of suspicion, early recognition through clinical evaluation and multimodal imaging are essential for timely management. Despite a generally poor prognosis, novel therapeutic modalities appear promising in improving both quality of life and life expectancy in cancer patients. Continued collaborative research and a multidisciplinary management strategy between ocular oncologists, radiation oncologists, and medical oncologists will lead to a better understanding of tumor biology and optimize patient outcomes.
Introduction: Retinoblastoma (Rb) is a rare but aggressive pediatric eye cancer. This study aimed to examine its epidemiological characteristics, diagnostic stages, and survival factors in Kazakhstan, where national-level data have previously been limited. Methods: A retrospective national registry study was conducted using data from the Scientific Center for Pediatrics and Pediatric Surgery, the country's referral center for Rb (2015-2024). Incidence was calculated using a birth cohort approach per 100,000 live births. Demographic and clinical characteristics, including diagnostic intervals (lag time 1: symptom onset to diagnosis; lag time 2: diagnosis to treatment initiation), were analyzed. Survival was estimated using Kaplan-Meier methods. Prognostic factors were assessed using univariate and multivariable Cox proportional hazards regression. Results: A total of 167 cases were recorded. The cumulative birth cohort incidence was 4.18 per 100,000 live births (1:23,915). Median age at diagnosis was 14 months (IQR: 6.5-27.5). Unilateral disease occurred in 78.4% of patients, and 76.9% were diagnosed at advanced stages (D or E). Extraocular involvement was observed in 9.8% of affected eyes. Hereditary Rb accounted for 6% of cases. Median lag time 1 was 60 days (IQR: 30-120), and median lag time 2 was 12 days (IQR: 7-20). Overall mortality was 11.4%, with most deaths occurring within 18 months. Older age at diagnosis (adjusted HR: 1.031; 95% CI: 1.008-1.054; p = 0.009) and longer lag time 2 (adjusted HR: 1.006; 95% CI: 1.004-1.008; p < 0.001) were independently associated with increased mortality. Conclusion: Rb in Kazakhstan remains characterized by a high proportion of advanced-stage disease and measurable early mortality. Delays in treatment initiation and older age at diagnosis independently predict poorer survival, underscoring the need to strengthen early detection and optimize care pathways.
Background:Iridocyclectomy still remains one of the main treatment options for intraocular anterior tumors. This surgical approach has undergone a century of development. Many famous ocular oncologists have contributed substantially to the advancement of the surgical removal of iridociliary tumors. Summary:The present article offers a historical overview of this surgical modality, surveys the evolution of techniques and approaches, and summarizes measures aimed at improving the efficacy and safety by reducing complication rates and managing intra- and postoperative adverse events. Special attention will be given to a contribution made by Prof. Leonid F. Linnik (1930-2008), a major Russian ocular oncologist. Key Messages:Although block excision continues to dominate contemporary practice, a variety of unresolved challenges necessitate further technological innovation.
Background: Retinal vasoproliferative tumors (RVPTs) are rare, benign lesions appearing as elevated, pink masses in the peripheral retina. Initially considered acquired retinal capillary hemangioblastomas, RVPTs are now recognized as distinct entities, with idiopathic and secondary forms. Though primarily affecting individuals between 30 and 50 years of age, their pathogenesis remains under investigation. Summary: Recent histopathological evidence suggests RVPTs have a predominantly glial rather than vascular origin. Clinically, RVPTs cause visual deterioration, floaters, and photopsia, often with subretinal/intraretinal exudation, epiretinal membranes, vitreous hemorrhage, or retinal detachment. Fluorescein angiography reveals telangiectatic vessels with intense late-phase hyperfluorescence. Secondary RVPTs comprise up to 84% of cases, linked to conditions such as Coats’ disease, uveitis, and toxoplasmosis. Management depends on tumor size, location, and complications. Small, asymptomatic lesions may be observed, while vision-threatening cases require intervention. Treatment options include cryotherapy, laser photocoagulation, photodynamic therapy, intravitreal anti-vascular endothelial growth factor/corticosteroid injections, plaque brachytherapy, and vitreoretinal surgery. While some tumors remain stable without treatment, surgical interventions, particularly pars plana vitrectomy, effectively control complications and tumor activity. Key Messages: The evolving understanding of RVPT pathogenesis necessitates multicenter studies to establish standardized diagnostic and therapeutic guidelines. Integrating histopathological insights with clinical findings will optimize management strategies and improve patient outcomes for these rare retinal tumors.
Introduction:Choroidal melanoma is the most common primary intraocular malignancy in adults. Early detection through ophthalmic screening increases the likelihood of globe-preserving treatment and may reduce metastasis while maximizing survival. Racial and ethnic disparities in access to care may influence outcomes, metastasis, and mortality. Methods:This retrospective cohort study used data from 2004 to 2025 within a federated electronic health record database. Adult patients (≥18 years) with choroidal melanoma were identified. Race/ethnicity was categorized as non-Hispanic white (NHW) or Black/Hispanic. The primary outcomes were primary enucleation, development of liver metastasis, and all-cause mortality within 1, 3, or 5 years from the time of choroidal melanoma diagnosis. Propensity score matching (PSM) was performed to balance cohorts. Multivariate Cox proportional hazards models were used to identify factors associated with enucleation, metastasis, and mortality. Results:A total of 17,436 patients with choroidal melanoma were identified, of whom 659 (6.3%) were Black/Hispanic and 9,883 (93.7%) were NHW. After PSM, primary enucleation within 90 days of diagnosis occurred in 5.2% of Black/Hispanic patients versus 3.8% of NHW patients (risk ratio [RR] 1.36, 95% confidence interval [CI] 0.821-2.253, p = 0.2305). Cumulative rates of liver metastasis in the Black/Hispanic group were significantly higher than NHW at 1 year (RR 2.42, CI: 1.336-4.387), 3 years (RR 1.96, CI: 1.246-3.083), and 5 years (RR 1.77, CI: 1.195-2.611) (p < 0.01 for each). All-cause mortality was higher in the Black/Hispanic cohort at 1 year (RR 1.96, CI: 1.212-3.163), 3 years (RR 1.75, CI: 1.249-2.453), and 5 years (RR 1.48, CI: 1.105-1.976) (p < 0.01 for each). On multivariate Cox analysis, male sex (hazard ratio [HR] 1.49, CI: 1.275-1.736, p < 0.0001) was associated with higher risk of enucleation, while a history of choroidal nevus was protective (HR 0.59, CI: 0.424-0.831, p = 0.0024). For metastatic risk, Black/Hispanic race/ethnicity (HR 1.54, CI: 1.242-1.904) and enucleation (HR 3.27, CI: 1.845-5.780) were associated with an increased risk, whereas prior nevus was protective (HR 0.43, CI: 0.324-0.577). All-cause mortality was elevated with older age (HR 1.03, CI: 1.021-1.031), male sex (HR 1.22, CI: 1.084-1.373), Black/Hispanic race/ethnicity (HR 1.42, CI: 1.135-1.769), metastatic liver disease (HR 7.49, CI: 6.051-9.269), among other comorbidities. Prior nevus history conferred a survival benefit (HR 0.59, CI: 0.458-0.762). Conclusion:In patients with newly diagnosed choroidal melanoma, Black/Hispanic patients had higher rates of metastatic disease and mortality. Previous history of choroidal nevus was associated with a lower risk of primary enucleation, metastatic disease, and mortality.
Introduction: Iris and ciliary body melanomas are rare but potentially life-threatening tumors that are difficult to distinguish from benign nevi. Earlier diagnosis improves outcomes, but reliable clinical indicators are limited. Glaucoma has been observed in conjunction with iris or ciliary body melanoma, but its prevalence and clinical significance at a population level are not well established. Our objective was to determine the prevalence of glaucoma in iris and ciliary body melanoma compared with nevus and to assess whether unilateral glaucoma may serve as a diagnostic indicator of melanoma. Methods: This retrospective cohort study used the IBM MarketScan Research Database, which contains de-identified longitudinal healthcare claims data from commercially insured patients in the USA. Patients with iris or ciliary body melanoma or nevus were identified by International Classification of Diseases, Tenth Revision (ICD-10) diagnostic codes. Outcomes included the prevalence of glaucoma prior to treatment in melanoma versus nevus, age-stratified prevalence of glaucoma, and rates of glaucoma surgery. Odds ratios (ORs) with 95% CIs were calculated to compare groups. Results: A total of 17,978 patients were included (112 with melanoma, 17,866 with nevus). Glaucoma prevalence prior to treatment was significantly higher in the melanoma cohort than nevus cohort (11.6% vs. 1.3%; OR: 10.1; 95% CI: 5.1-18.4; p < 0.001). This pattern persisted across all age groups, with the greatest relative difference observed in patients aged 20-39 years (10.9% vs. 0.7%; OR: 14.2; 95% CI: 1.5-63.0; p = 0.01). Among patients with glaucoma, surgery was required more frequently in the melanoma cohort (18.9%) than nevus cohort (15.0%). Conclusion: Unilateral glaucoma is significantly more common in eyes with iris and ciliary body melanoma than in those with iris and ciliary body nevus in this claims-based cohort, and melanoma patients are more likely to require surgical management. Unilateral glaucoma may serve as an important early clinical indicator of melanoma, although further clinical correlation is necessary. Incorporating glaucoma status into diagnostic criteria could improve recognition, prompt referral and biopsy, and reduce delays in treatment.
Introduction: The aim of this study was to describe the clinical features, multimodal imaging characteristics, treatment outcomes, and prognostic factors in patients with diffuse choroidal hemangioma (DCH) associated with Sturge-Weber syndrome (SWS)/phakomatosis pigmentovascularis (PPV) at a referral center in India. Methods: A retrospective observational study was conducted between 2013 and 2024 on patients diagnosed with SWS/PPV and associated DCH. A poor outcome was defined as a final best-corrected visual acuity (BCVA) of 20/200, persistent or recurrent exudative retinal detachment, persistent tumor, or disease recurrence. Results: A total of 29 patients (32 eyes) with SWS/PPV-associated DCH were included. The mean age at presentation was 25.3 ± 11.6 years, with a female predominance (59%). Most patients had SWS (90%), while 10% had PPV. Exudative retinal detachment and glaucoma were observed in 46% and 31% of SWS eyes, and 66% and 83% of PPV eyes, respectively. The mean tumor basal diameter and thickness were 8.31 mm and 4.16 mm at presentation. Selected cases were managed with plaque brachytherapy, trabeculectomy, external beam radiotherapy, or intravitreal anti-VEGF injections. Complete tumor regression was achieved in 22% of eyes. Mean BCVA improved significantly from 20/1200 to 20/160 at final follow-up; however, a poor visual outcome occurred in 69% of eyes. On multivariate analysis, persistent tumor (p = 0.008) and tumor recurrence (p = 0.03) were independent predictors of poor outcome. Conclusion: DCH in SWS is a vision-threatening condition requiring individualized multimodal treatment. Although anatomical and visual improvement is achievable, recurrence and tumor persistence are common and predict poor outcomes. Thus, long-term follow-up and early intervention are critical for optimal management.
Introduction:Retinoblastoma provides an important opportunity to assess how racial and socioeconomic disparities in health care access may affect the timely diagnosis and treatment of a life-threatening disease. Methods:Patients with retinoblastoma treated at a single institution (1999-2021) were retrospectively reviewed with respect to race, Zone Improvement Plan (ZIP) code, and International Intraocular Retinoblastoma Classification (IIRC) Grouping. Socioeconomic disadvantage score (SDS) was assigned based on census data from each ZIP code. Logistic regression was performed, and odds ratio (OR) and 95% confidence intervals (CIs) were calculated using the generalized estimating equations (GEEs) logistic regression model (SAS, version 9.4) to analyze the probability of locally advanced retinoblastoma with respect to race and SDS. Advanced retinoblastoma was defined as IIRC Group E disease in at least one eye. Results:Of 445 patients, 189 presented with group E disease in at least one eye. Locally advanced retinoblastoma positively correlated with non-White race (OR = 1.38, 95% CI [1.03-1.85]). The SDS demonstrated a positive trend with advanced disease but did not reach statistical significance (OR = 3.03, 95% CI [0.25-36.27]). When accounting for age at presentation, neither race (OR = 1.13, 95% CI [0.74-1.72]) nor SDS (OR = 4.05, 95% CI [0.23-71.96]) were significantly associated with advanced retinoblastoma. Conclusions:A positive association was found between non-White race and Group E retinoblastoma, though this was not statistically significant after accounting for age at diagnosis. This emphasizes the importance of continuing education and outreach efforts to all patient communities to facilitate earlier diagnosis and ensure that care is delivered as equitably as possible.
Background: This review explores the development and legacy of the Reese-Ellsworth classification system for retinoblastoma, a landmark achievement in pediatric oncology. Summary: We first highlight the history of cancer staging in the early 20th century. Then, we discuss the lives of Algernon B. Reese and Robert M. Ellsworth, two pioneering figures in ophthalmic oncology, and their development of the Reese-Ellsworth classification system in 1963. The system served as the international standard for over 3 decades, stratifying patients by prognosis and enabling standardized treatment protocols and collaborative research that contributed to dramatic improvements in retinoblastoma survival rates. However, as the standard of care shifted from radiation to chemotherapy in the latter half of the 20th century, the system became obsolete and was ultimately replaced. Key Messages: The story of Reese and Ellsworth exemplifies how disease staging systems must adapt to changing therapeutic paradigms while demonstrating the importance of standardized classification in advancing cancer research.
Introduction: Extrascleral extension (EOE) of uveal melanoma is a known negative prognostic factor, more frequently observed in medium to large tumors. Although EOE is typically limited in size, extensive cases present significant challenges in treatment planning, and the optimal management approach remains controversial. This study aimed to estimate the incidence of EOE in patients with uveal melanoma and analyze the prognosis of patients treated at the Italian Hospital of Buenos Aires from 2007 to June 2024. Methods: We conducted an observational, analytical, retrospective cohort study from 2007 to June 2024. Patients with EOE at diagnosis or during follow-up were identified. Epidemiological characteristics, tumor dimensions, timing of EOE, metastasis occurrence, and survival data were analyzed. Results: A total of 181 patients diagnosed with uveal melanoma were included, of whom twelve (6.62%) presented EOE, and 54.5% were male. The mean age was 64.41 years (SD 14.39). Six patients (50%) had EOE at diagnosis (average tumor thickness 9.51 mm, average diameter 17.7 mm), while six developed it during follow-up. The median time to event was 31.5 months (range: 8-120 months). Among these 12 patients, three developed metastases within the first year after diagnosis and treatment. Conclusions: EOE is a poor prognostic factor and tends to occur in larger tumors. It may be present at diagnosis or appear years later, reinforcing the need for long-term surveillance. Early identification is crucial to ensure appropriate treatment.
