Introduction: Ocular and ocular-adnexal lymphomas comprise biologically distinct entities, and the population-level incidence of a later lymphoma is uncertain. Methods: This retrospective cohort study used the Surveillance, Epidemiology, and End Results 17 registries (excluding Alaska), November 2023 submission. Persons with a first primary malignant lymphoma of the eyelid, conjunctiva/cornea, retina/uvea, lacrimal gland, orbit, or overlapping/unspecified eye site diagnosed in 2000–2011 were followed through 2021, beginning 2 months after diagnosis. This pragmatic exclusion was chosen to reduce counting of events documented during the initial staging period, although it cannot eliminate occult synchronous disease. Standardized incidence ratios (SIRs), exact Poisson 95% confidence intervals (CIs), and excess incidence per 10,000 person-years were calculated for subsequent registry-coded Hodgkin or non-Hodgkin lymphoma. Poisson likelihood-ratio tests assessed heterogeneity by index site and latency. Results: The cohort included 2,256 persons contributing 24,779.04 person-years. A total of 101 subsequent lymphoma events occurred versus 16.48 expected (SIR, 6.13; 95% CI, 4.99–7.45; excess incidence, 34.11 per 10,000 person-years). At the broad histologic-recode level, 97 events were non-Hodgkin lymphoma and 4 were Hodgkin lymphoma; 97 events followed an index B-cell non-Hodgkin lymphoma and 4 followed an index lymphoid neoplasm not otherwise specified. Site heterogeneity was not established (p = 0.104), and relative incidence was similar across the 2–11, 12–59, 60–119, and 120-month-or-longer latency bands (p = 0.993). Conclusion: A first primary ocular or ocular-adnexal lymphoma was followed by a persistent approximately sixfold excess of subsequent registry-coded lymphoma. This population-level ratio is not an individual probability and does not establish that the events were independent second primary lymphomas. The signal supports long-term, histology-informed multidisciplinary vigilance, but not a specific surveillance protocol.
Purpose: To evaluate the choroidal vascularity index (CVI) in eyes with choroidal nevi (CN) and compare it to the corresponding anatomical region in the fellow eye, in order to assess whether the presence of a CN alters the vascular composition of the adjacent choroid. Methods: This cross-sectional study included patients with unilateral CN imaged with enhanced depth imaging optical coherence tomography (EDI-OCT). Foveal and perilesional CVI was calculated using a standardized binarization and segmentation protocol based on previously published methods and compared with the homologous region of the fellow eye. Associations between CVI and demographic, ocular, and nevus-related characteristics were also assessed. Results: A total of 64 patients (64 eyes with CN) were included. Perilesional CVI did not differ between the CN eye and the corresponding region of the fellow eye (66.4 ± 4.8% vs 66.5 ± 4.6%, p = 0.682), nor did it vary by anatomical location. Foveal CVI was similarly comparable between the CN eye and the fellow eye (68.8 ± 3.4% vs 68.4 ± 3.1%, p = 0.212). Neither foveal nor perilesional CVI was associated with age, sex, subfoveal choroidal thickness, or any nevus characteristic tested, including largest basal diameter, thickness, configuration, drusen, subretinal fluid, or choroidal neovascularization. Conclusions: The presence of a CN does not appear to alter the vascular composition of the adjacent or foveal choroid. These findings support CVI as a reproducible parameter in eyes with benign choroidal lesions and provide a baseline against which future studies of indeterminate melanocytic lesions and choroidal melanoma may be compared.
Introduction This study examined associations between race/ethnicity, geographic classification, and median household income with treatment allocation as well as treatment initiation among pediatric patients with orbital embryonal rhabdomyosarcoma (RMS) in the U.S. Methods We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results (SEER) program, which includes 18 registries representing approximately 46% of the U.S. population. Children <15 years of age diagnosed between 2000 and 2021 with orbital embryonal RMS were included. Outcomes included receipt of surgery, radiation therapy (RT), and chemotherapy. Binary logistic regression models provided adjusted odds ratios (aORs) with 95% confidence intervals (CIs) for treatment option receipt. Multiple linear regression assessed predictors of time to treatment initiation. Results Of 113 eligible patients, 67 (59.29%) were Non-Hispanic White, 102 (89.16%) resided in metropolitan counties, and 93 (82.69%) were from counties with median household income ≥$60,000. Most patients received chemotherapy (n = 108, 95.58%) and RT (n = 102, 90.27%), and 59 (52.21%) patients underwent surgery. Non-Hispanic White patients had higher odds of receiving RT than Non-White patients (aOR: 5.328; 95% CI: 1.163–14.403; P = 0.009). Chemotherapy was more accessible to patients residing in metropolitan counties (aOR: 14.811; 6.220–25.920; P < 0.001). Age at diagnosis was a significant predictor of time to treatment (P = 0.017). Age at diagnosis was distributed as follows: <1 year (n = 4, 3.54%), 1–4 years (n = 35, 30.97%), 5–9 years (n = 48, 42.48%), and 10–14 years (n = 26, 23.01%). Conclusion Ethnoracial and geographic disparities were observed and may affect access to RT and chemotherapy in children with orbital embryonal RMS. Age at diagnosis was a significant predictor of time to treatment initiation, a finding that warrants further investigation. Efforts to address structural barriers to equitable multimodal care are needed.
Purpose: To assess the immunohistochemical (IHC) and molecular/genetic profiles of eyelid sebaceous gland carcinoma (SGC) and to examine correlations between clinical features and IHC and molecular/genetic alterations. Methods: This narrative review identified published studies reporting IHC and molecular/genetic findings in eyelid SGC. Eligible studies were reviewed, and extracted data included demographic characteristics, clinical and histopathologic tumor features, IHC biomarker expression, genetic and molecular alterations, and evaluated correlations between IHC or molecular findings and clinical behavior. Results: A total of 38 studies published between 2000 and 2025 were included, encompassing 1564 patients with eyelid SGC. Lymph node invasion or metastasis was reported in 15.9% of cases, and the mortality rate was 6.3%. Among diagnostic biomarkers, adipophilin was identified as a reliable marker for distinguishing SGC from other periocular neoplasms. With respect to tumorigenesis, multiple molecular pathways were implicated, including dysregulation of cholesterol metabolism, aberrant retinoic acid receptor expression, EGFR (epithelial growth factor receptor) overexpression, and androgen receptor signaling. Regarding tumor invasion, metastatic potential, and survival, associations involving Sonic hedgehog (SHH) signaling, the miR-200 family, and epithelial–mesenchymal transition (EMT)–related markers (ZEB2, vimentin, and E-cadherin) appeared to play critical roles. Conclusion: This review highlights adipophilin as a valuable diagnostic marker for eyelid SGC, particularly in diagnostically challenging cases. In addition, androgen-related pathways and EMT-associated mechanisms appear to contribute significantly to both the pathogenesis and aggressive behavior of eyelid SGC. These pathways warrant further investigation, especially for their potential relevance in the development of future targeted therapeutic strategies.
Introduction: Intraocular medulloepithelioma (IOME) is a rare embryonal tumour of the non-pigmented ciliary epithelium that predominantly affects children. Adult-onset disease is exceedingly uncommon. Delayed diagnosis may result in local invasion, recurrence, and mortality. Case presentation: We report two cases of IOME occurring at opposite ends of the age spectrum, illustrating the diagnostic heterogeneity of this rare tumour. The first case involved a 53-year-old man with initially benign-appearing IOME, that subsequently demonstrated malignant features with orbital extension over two years, necessitating exenteration. After exenteration, there was locoregional recurrence and eventual disease-related mortality. The second case involved a 4-year-old boy whose IOME was initially misdiagnosed as persistent fetal vasculature for over three years. Multiple intraocular surgeries were performed before he developed secondary rubeotic glaucoma and a painful blind eye requiring evisceration. Subsequent orbital recurrence necessitated exenteration, and the patient eventually succumbed to the disease. Conclusion: These cases underscore the distinct clinical presentations and diagnostic challenges of IOME in adult and paediatric patients. They emphasise the importance of maintaining a high suspicion for malignancy in atypical or treatment-refractory cases, particularly before repeated intraocular intervention or destructive ocular procedures.
