Background: Uveal melanoma (UM) metastasis follows variable temporal trajectories, with recurrences ranging from rapid onset to decades post primary therapy. Outcomes after metastasis also vary significantly. Predicting high-risk “early” progressors from “late” recurring phenotypes would facilitate tailored approaches to management. Methods: A literature review was conducted to synthesise clinicopathologic and molecular predictors of metastatic latency and their impact on survival outcomes in patients with UM. Results: Early dissemination is associated with older age, male sex, larger basal dimensions, and ciliary body involvement. Genetically, monosomy 3, 8q gain and BAP1 loss drives a rapid-progression phenotype and poor survival. Conversely, SF3B1 mutations define a distinct late-onset cohort (median >6 years) with persistent lifetime risk. PRAME expression significantly accelerates metastatic kinetics, even in Gene Expression Profiling Class 1 tumours. While early recurrence correlates with dismal outcomes, late-onset disease is associated with extended post-metastasis survival and greater eligibility for interventions like liver-directed therapy. Key Message: Metastatic timing is related to biologic rather than stochastic determinants. Integrating molecular profiling with clinical staging and patient factors allows for more precise temporal risk stratification. These insights are essential for tailoring surveillance intensity and selecting candidates for emerging adjuvant therapies, such as tebentafusp and protein kinase C inhibitors.

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: 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.

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.

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.

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: 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: 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.

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.