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Purpose: To report the demographic profile, clinical presentation, and management outcomes of ocular surface squamous neoplasia (OSSN) treated with primary topical chemotherapy in a limited resource secondary eye care facility in rural parts of South India., Methods: Retrospective interventional study of 38 eyes of 37 patients with OSSN treated with topical 1% 5-Fluorouracil (5FU), over a period of two years., Results: The median age at presentation with OSSN was 44 years (mean, 46 years; range 13 to 74 years). Majority (76%) were males. The most common morphological variant was placoid OSSN (18, 47%). Limbus was the most common epicenter (31, 82%). Corneal OSSN was the most initially misdiagnosed variant (n = 3). Of the 38 eyes receiving one week on and 3-weeks off cycles of 5FU regimen, complete tumor resolution was achieved in 36 (95%) eyes. The median number of topical 5FU cycles for tumor resolution was 2 (mean, 2; range, 1 to 4). Over a median follow-up period of 5 months (mean, 6 months; range, 1 to 27 months), tumor recurrence was noted in 3 eyes (8%), of which one case had xeroderma pigmentosum with bilateral multifocal recurrence. Complication rate was 5% (n = 2), which included transient conjunctival hyperemia (n = 1), and bacterial keratitis (n = 1) which resolved with fortified antibiotics., Conclusion: Primary chemotherapy with topical 1% 5FU is a safe and effective management modality for OSSN at limited resource settings in rural India., (© 2024. The Author(s).)

Purpose: To describe the epidemiology, incidence, mortality and survival of ocular cancer in Cali between 1962 and 2019., Methods: Ecological population-based study analyzing data of incidence, mortality, and 5-years survival of malignant ocular tumors from the Populational Cancer Registry of Cali between 1962 and 2019., Results: Between 1962 and 2019, 586 ocular tumors were found, 50.5% occurred in females, the mean age at diagnosis was 45 years (standard deviation = 25), 70.3% of ocular malignancies occurred in >14 years. The average annual incidence rate was 7.8 per million for male and 6.9 per million for females. Retinoblastoma (21%), squamous cell carcinoma (20%), melanoma (16%) and lymphoma (8%) were the most common neoplasm. In those <15 years, the most frequent malignant tumors were retinoblastomas (85.7%), followed by non-specified malignant neoplasm (NOS, 7.9%), and rhabdomyosarcoma (3.6%). In those >14 years, there were NOS (30%), followed by squamous cell carcinomas (28%), melanomas (23%), and lymphomas (9.7%). Conjunctiva (38.2%), retina (21%) and orbit (10%) constituted the majority of anatomical sites of ocular tumors. The survival rate was about 83.2% and mortality did not show a decreasing trend over time ( p  > .05)., Conclusions: The incidence of ocular cancer in Cali has a slightly increasing trend, with stable behavior in the last decades. Squamous cell carcinoma, retinoblastoma, melanoma and lymphoma are the most frequent ocular cancers, with being retinoblastoma more frequent than melanoma. In general, ocular cancer had good survival rates in Cali.

Purpose: To investigate lacrimal gland malignancies' incidence and survival rates in the USA between 1995 and 2018., Methods: Incidence and survival data from the North American Association of Central Cancer Registries of lacrimal gland malignancies between 1996 and 2018 were extracted and analyzed., Results: This study analyzed data pertaining to 3620 patients (females 56.7%, n  = 2051). A sizable number of patients were 60-79 years of age (45.1%, n  = 1633), with a substantial majority being of the non-Hispanic white ethnicity (82.9%, n  = 3002). A little above half of patients (52.1%, n  = 1886) had presented with a localized disease. Lymphomas represented 59.3% ( n  = 2146) of lacrimal gland malignancies, while 37% ( n  = 1339) were carcinomas. The cumulative age-adjusted incidence rate per million was 0.53 for all malignancies, 0.31 for lymphomas, and 0.2 for carcinomas. While the annual crude incidence rate showed a significant steady increase (average annual change of 1.24%; P  < .05), age-adjusted rates did not show a similar trend. The five- and 10-year relative survival rates were 88.64 months (95% confidence interval (CI): 85.81-90.93) and 80.26 months (95%CI: 76.21-83.7), respectively. Older age, non-lymphoma tumors, and advanced stage at diagnosis were significantly associated with worse outcomes. Relative survival rates did not show significant changes from 1995 to 2018, irrespective of gender, race or treatment received., Conclusions: Early detection and localized tumor management can improve survival outcomes for patients with lacrimal gland malignancies. Further research is needed to understand these malignancies' risk profiles and develop more effective treatment strategies.

Purpose: To assess the prevalence and subtypes of Human Papillomavirus (HPV) in Ocular Surface Squamous Neoplasia (OSSN) in Human Immunodeficiency Virus (HIV) positive and negative patients in South Africa., Basic Procedures: This study was a single center retrospective cross-sectional study, conducted at Tygerberg Hospital, Western Cape, South Africa. We assessed 63 histopathologically confirmed OSSN formalin-fixed paraffin-embedded (FFPE) tissue blocks from 2015-2023. The presence of HPV was determined using the Hybrispot Direct Flow Chip Kit. Corresponding clinical data was retrieved from the National Health Laboratory Service (NHLS) central data warehouse., Main Findings: Of the confirmed OSSN samples, 66.7% tested positive for HPV (95% confidence interval [CI] 54-77.3%). Of the 42 HPV positive samples, 38 (90.5%) had one or more known genotypes detected and 4 had unknown genotypes. The most prevalent subtypes were HPV 11, 16 and 18 (found in 61.9%, 52.4% and 33.3% of HPV positive samples respectively). 88.9% of the lesions biopsied were from HIV positive patients, of whom 56.4% had a CD4 + count of < 200 cells/µL. A lower median CD4 + count was detected among HIV positive patients with invasive squamous cell carcinoma compared to those with moderate dysplasia (p < 0.0198)., Conclusions: There is a high prevalence of HPV in OSSN in South Africa. Certain subtypes namely, 11, 16, 18, 31, 33 and 35 may be more carcinogenic. HIV with HPV co-infection may be linked as a causative factor in the development of OSSN., (© 2024. The Author(s).)

Background/objectives: Ocular melanoma is a rare, but deadly cancer. This large cancer registry study examines the associations between solar ultraviolet radiation (UVR) and incidence of different anatomical sites of ocular melanoma by sex, age, laterality, and race and ethnicity., Methods: Incidence data were derived from 21 cancer registries in the US for the years 2000-2019. Satellite-based UVR estimates were linked to county of residence at diagnosis. Incidence rate ratios (IRRs) and 95% confidence intervals (CIs) were calculated for UVR quartiles using Poisson models., Results: UVR was not associated with total ocular melanoma (N = 18,089) comparing Q4 versus Q1 (IRR = 0.98; 95%CI:0.94,1.03; p-trend = 0.07) or conjunctival melanoma (IRR = 0.99; 95%CI:0.82,1.19; p-trend = 0.81). However, in analyses of continuous UVR (per 10 mW/m 2 ), risks were reduced for total ocular melanoma (IRR = 0.97; 95% CI: 0.96, 0.99). Incidence was increased for ciliary body/iris melanoma in the highest UVR quartile (IRR = 1.63; 95%CI:1.43,1.87; p-trend < 0.0001) and remained increased in non-Hispanic White individuals only. Incidence was reduced for choroidal melanoma in the highest UVR quartile (IRR = 0.86; 95%CI:0.82,0.91; p-trend < 0.0001)., Conclusions: UVR may be associated with increased risk of ciliary body/iris melanoma. Reduced risk of choroidal melanoma may be due to higher diffuse UVR exposure to posterior ocular sites in locations at higher latitudes. Our results support and expand previous findings of associations of UVR using various surrogates on ocular melanoma risk and serve as a starting point for understanding the differences in the relationship between UVR and specific anatomical sites., (© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Background: This study aims to investigate the global disease burden, risk factors, and temporal trends of eye cancer by sex and age group., Methods: Databases including Cancer Incidence in Five Continents volumes I-XI, the Nordic Cancer Registries, the Surveillance, Epidemiology, and End Results Program and the WHO IARC mortality database were accessed to extract incidence and mortality data. Joinpoint regression analyses were conducted to evaluate the Average Annual Percentage Change of the incidence and mortality., Results: The age-standardised rates of eye cancer incidence and mortality were 0.49 and 0.08 globally in 2020. Higher incidence rates were observed in Sub-Saharan Africa (ASR = 4.06), Western Europe (ASR = 0.89), and Northern Europe (ASR = 0.84), but higher mortality was observed only in Sub-Saharan Africa (ASR = 1.59). Lower HDI, higher prevalence of UV exposure and lower prevalence of several lifestyle habits and metabolic syndromes were associated with higher incidence and mortality. There was an overall stable incidence trend and a decreasing mortality trend. Notably, all countries reporting decreasing trend in mortality were in the Asian or European region., Conclusions: Although higher incidence was observed in both African and European regions, only the Sub-Saharan Africa region reported high mortality, indicating inequity in the access of healthcare and treatment resource. Higher prevalence of UV exposure was associated with both higher incidence and mortality. Education should be provided to increase the awareness of eye protection. An overall declining mortality trend was found, but it was limited to only Asian and European countries., (© 2024 The Authors. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Background: Prior works have studied the impact of social determinants on various cancers but there is limited analysis on eye-orbit cancers. Current literature tends to focus on socioeconomic status and race, with sparse analysis of interdisciplinary contributions. We examined social determinants as measured by the Centers for Disease Control and Prevention (CDC) Social Vulnerability Index (SVI), quantifying eye and orbit melanoma disparities across the United States., Methods: A retrospective review of 15,157 patients diagnosed with eye-orbit cancers in the Surveillance, Epidemiology, and End Results (SEER) database from 1975 to 2017 was performed, extracting 6139 ocular melanomas. SVI scores were abstracted and matched to SEER patient data, with scores generated by weighted averages per population density of county's census tracts. Primary outcome was months survived, while secondary outcomes were advanced staging, high grading, and primary surgery receipt., Results: With increased total SVI score, indicating more vulnerability, we observed significant decreases of 23.1% in months survival for melanoma histology (p < 0.001) and 19.6-39.7% by primary site. Increasing total SVI showed increased odds of higher grading (odds ratio [OR] 1.20, 95% confidence interval [CI] 1.02-1.43) and decreased odds of surgical intervention (OR 0.94, 95% CI 0.92-0.96). Of the four themes, higher magnitude contributions were observed with socioeconomic status (26.0%) and housing transportation (14.4%), while lesser magnitude contributions were observed with minority language status (13.5%) and household composition (9.0%)., Conclusions: Increasing social vulnerability, as measured by the CDC SVI and its subscores, displayed significant detrimental trends in prognostic and treatment factors for adult eye-orbit melanoma. Subscores quantified which social determinants contributed most to disparities. This lays groundwork for providers to target the highest-impact social determinant for non-clinical factors in patient care., (© 2024. Society of Surgical Oncology.)

This epidemiological study examined ocular and orbital lymphomas in the United States from 1995 to 2018, using data from the North American Association of Central Cancer Registries database of 87,543 patients with ocular and adnexal malignancies. We identified 17,878 patients (20.4%) with ocular and orbital lymphomas, with an age-standardized incidence rate (ASIR) of 2.6 persons per million (ppm). The incidence was the highest in the orbit (ASIR = 1.24), followed by the conjunctiva (ASIR = 0.57). Non-Hodgkin B-cell lymphoma was the most prevalent subtype (85.4%), particularly marginal-zone lymphoma (45.7%). Racial disparities were noted, with Asia-Pacific Islanders showing the highest incidence (orbit, 1.3 ppm). The incidence increased significantly from 1995 to 2003 (Average Percent Change, APC = 2.1%) but declined thereafter until 2018 (APC = - 0.7%). 5-year relative survival (RS) rates varied, with the highest rate for conjunctival lymphoma (100%) and the lowest for intraocular lymphoma (70.6%). Survival rates have generally improved, with an annual increase in the 5-year RS of 0.45%. This study highlights the changing epidemiological landscape, pointing to initial increases and subsequent decreases in incidence until 2003, with survival improvements likely due to advancements in treatment. These findings underscore the need for further research to investigate the root causes of these shifts and the declining incidence of ocular lymphoma., (© 2024. The Author(s).)

Background: Studies on the epidemiological information and prognosis of primary malignant lacrimal gland tumors (MLGTs) are rare for its low occurrence. The goal of our research was to investigate the epidemiological characteristics and survival outcomes of patients with MLGTs., Methods: Incidence and demographic information of patients with MLGTs were collected from the Surveillance, Epidemiology, and End Results (SEER) database. To identify independent prognostic factors for disease-specific survival (DSS) and overall survival (OS), univariate and multivariate Cox regression analysis were performed., Results: The overall incidence of primary MLGTs from 1975 to 2020 was 0.413/1,000,000 (according to the 2000 American standard population), with a steadily increasing incidence over years. A total of 964 patients with primary MLGTs were diagnosed, with an average age of 59.3 years. Of these, 53.2% were aged ≥60 years, 57.4% were female, and 77.1% were whites. Multivariate Cox regression analysis demonstrated that year of diagnosis, age, sex, histological type, SEER stage, surgery, and chemotherapy were independent prognostic factors of DSS or OS., Conclusions: Although primary MLGT is rare, its incidence has steadily increased in the past 46 years, and surgery was related to a better prognosis., (© 2024 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Ocular melanoma is a rare but complex disease in current medical practice. Our retrospective study spans over a period of 28 years and analyzed uveal and conjunctival melanomas that were consecutively admitted, diagnosed, and treated in the 2nd Ophthalmology Clinic of Prof. Dr. Nicolae Oblu Emergency Clinical Hospital, Iaşi, Romania. The patients were selected from the records of the Department of Pathology of our Hospital, being diagnosed by standard histopathological techniques. The aim of this study was to summarize the epidemiological and pathological aspects of uveal and conjunctival melanomas in Northeastern region of Romania. In our study, we did not notice a predilection of uveal and conjunctival melanoma to one particular gender. The most common histological subtypes of ocular melanomas were the heavily pigmented spindle cell subtype, followed by the epithelioid subtype. Our patients sought medical help in a timely manner, before the systemic invasion of the disease could develop.

This paper pioneers the exploration of ocular cancer, and its management with the help of Artificial Intelligence (AI) technology. Existing literature presents a significant increase in new eye cancer cases in 2023, experiencing a higher incidence rate. Extensive research was conducted using online databases such as PubMed, ACM Digital Library, ScienceDirect, and Springer. To conduct this review, Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines are used. Of the collected 62 studies, only 20 documents met the inclusion criteria. The review study identifies seven ocular cancer types. Important challenges associated with ocular cancer are highlighted, including limited awareness about eye cancer, restricted healthcare access, financial barriers, and insufficient infrastructure support. Financial barriers is one of the widely examined ocular cancer challenges in the literature. The potential role and limitations of ChatGPT are discussed, emphasizing its usefulness in providing general information to physicians, noting its inability to deliver up-to-date information. The paper concludes by presenting the potential future applications of ChatGPT to advance research on ocular cancer globally., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Alotaibi, Rehman and Hasnain.)

Aims: The aim of this study is to describe the demographic, presenting features and associated risk factors of ocular surface squamous neoplasia (OSSN) at a tertiary eye hospital in Johannesburg, South Africa., Methods: An interventional prospective study of patients presenting with conjunctival masses was conducted. An electronic questionnaire was completed to document demographic data, presenting history, and associated risk factors. A slit lamp examination and photos were used to document and describe the clinical features at presentation. Cases (OSSN) and controls (benign lesions) were determined by histology., Results: There were 130 cases and 45 controls. Median age was 44 years (IQR: 35-51) with an equal gender distribution in cases. The prevalence of HIV in cases was 74% and was strongly associated with OSSN (p < 0.001). Vascularisation, leukoplakia and pigmentation were clinical features that distinguished cases from controls. A fibrovascular morphology was strongly associated with a benign histology (p < 0.001), whereas leukoplakic and gelatinous morphologies were associated with OSSN. Conjunctival intra-epithelial neoplasia made up 82% of cases., Conclusion: Our study describes a sample of OSSN that is young and has no gender predisposition. The majority of cases presented with CIN lesions, rather than SCC reported in other African countries. HIV was the most significant risk factor in this study population., (© 2023. The Author(s).)

Introduction: Ocular surface squamous neoplasia (OSSN) and pterygia share risk factors and co-exist in only a minority of cases. Reported rates of OSSN in specimens sent as pterygium for histopathological analysis vary between 0% and nearly 10%, with the highest rates reported in countries with high levels of ultraviolet light exposure. As there is a paucity of data in European populations, the aim of this study was to report the prevalence of co-existent OSSN or other neoplastic disease in clinically suspected pterygium specimens sent to a specialist ophthalmic pathology service in London, United Kingdom., Methods: We performed a retrospective review of sequential histopathology records of patients with excised tissue submitted as suspected "pterygium" between 1997 and 2021., Results: In total, 2061 specimens of pterygia were received during the 24-year period, with a prevalence of neoplasia in those specimens of 0.6% (n = 12). On detailed review of the medical records of these patients, half (n = 6) had the pre-operative clinical suspicion of possible OSSN. Of those cases without clinical suspicion pre-operatively, one was diagnosed with invasive squamous cell carcinoma of the conjunctiva., Conclusion: In this study, rates of unexpected diagnoses are reassuringly low. These results may challenge accepted dogma, and influence future guidance for the indications for submitting non-suspicious pterygia for histopathological analysis., (© 2023. The Author(s).)

Introduction: SCC (squamous cell carinomas) are among the most common eye neoplasms in horses. In recent studies Haflinger horses with a homozygous genotype for a missense variant in the DDB2 gene (damage specific DNA binding protein 2) had a significant increased risk of developing ocular SCC. The aims of this study were to determine the frequency of the SCC-associated risk allele in the DDB2 gene in Swiss and Austrian Haflinger populations and to validate the previously described phenotypic correlation. For this purpose, Haflingers presented at various horse clinics in Switzerland (n = 21, including 11 SCC cases), privately kept Haflingers (n = 52, including 1 SCC case), and Haflingers from a stud farm in the Austrian Tyrol (n = 53) were recruited. The individual DDB2 genotype of the animals was determined using a polymerase chain ceaction (PCR) test using hair follicle or whole blood samples. Of the 12 horses suffering from SCC, nine had ocular SCC and three had non-ocular SCC. Six of the nine Haflingers with ocular SCC and one of the three Haflingers with non-ocular SCC were homozygous for the DDB2 variant. Of the 113 clinically normal animals, 7/113 were homozygous (6 %) and 42/113 were heterozygous (37 %), which corresponds to an allele frequency of 24,8 % in the control cohort. The risk of ocular SCC occurring in Haflingers is significantly increased with the homozygous DDB2 genotype. However, not all animals with SCC carry this gene variant and not all DDB2 homozygous animals develop SCC, which can be explained by the multifactorial genesis of the disease. Due to the high frequency of the undesirable allele, we recommend taking the individual DDB2 genotype of breeding animals into account in order to avoid homozygous offspring with a greatly increased SCC risk by excluding high-risk matings.

Background/aims: Extranodal marginal zone lymphoma of ocular adnexa (OA-EMZL) is the most frequent type of ocular adnexal lymphomas, with a high rate of disease recurrence. Precise patient stratification based on disease recurrence is understudied. This study aims to identify risk factors of distant recurrence (DR) and local recurrence (LR) to construct a prognostic model optimising rapid decision of therapeutic strategies., Methods: A total of 104 patients diagnosed with OA-EMZL between January 2011 and February 2020 were enrolled. Propensity score matching was performed for DR and LR groups. A nomogram was generated using a multivariate Cox proportional hazards model., Results: After matching, different independent risk factors of DR and LR were identified. Monocyte percentage (p=0.015) and M category >0 (p=0.043) were significant independent risk factors of DR. Epiphora (p<0.001) was the significant independent risk factor of LR. Three factors (monocyte percentage, M category >0, age >60) were integrated into the nomogram to predict the risk of DR. It had a relatively better discriminative ability for distant recurrence-free survival (C-index: 3-year, 0.784; 6-year, 0.801) than IPI score (C-index: 3-year, 0.663; 6-year, 0.673) in the cohort of all patients., Conclusion: Our analyses suggested DR and LR as two distinct prognostic events, and additionally identified novel risk factors of them. The nomogram may serve as a practical tool for the prognostic estimation and rapid decision of therapeutic strategies for patients with OA-EMZL., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: The authors have no conflicts of interest to disclose.

Introduction: Oculo-orbital tumors are common. Their clinical and histological features are multiple. The management of oculo-orbital tumors is a real challenge in sub-Saharan Africa, especially in our context. The aim of this study was to contribute to the improvement of the management of oculo-orbital tumors at the Bangui Teaching Hospital (CNHUB)., Methodology: This was a prospective, observational study of oculo-orbital tumors over 12 months from January 1 to December 31, 2022 in the ophthalmology department of the CNHUB. All consenting patients with histologically proven tumors of the eyeball and/or its adnexa were included in this study, regardless of age and sex.The parameters studied were:epidemiological: age, sex, occupation, origin;clinical: time of consultation, functional signs and associated signs, physical examination signs, risk factors;paraclinical: report of the anatomopathological examination of the operating room;therapeutic: treatment modalities (chemotherapy, surgery, palliative treatment, radiotherapy).These data were collected on a pre-established survey form for direct administration. The processing and the production of the results were carried out with Excel and Epi Info 7.0 software., Results: We included 97 patients with a mean age of 37.5 years, ranging from 2 to 70 years. There was a male predominance (sex-ratio = 1.8). Almost all patients lived in Bangui (96%). Patients under 40 years of age were most commonly affected (55%). The majority of tumors were benign (55%). Ocular pain (24%), lacrimation (22%) and orbital swelling (16%) were the main symptoms. Risk factors were mainly age (23%), diabetes (22%), HIV infection (16%) and alcohol and tobacco use (37%). The most common treatments were surgical removal (68%) and enucleation (16%). Chemotherapy was combined with surgery in 37% of cases., Conclusion: Oculo-orbital tumors are frequent in our context. Their morpho-histological presentations are varied. A multidisciplinary approach is important for a good management., (Copyright © 2023 SFMTSI.)

Investigating secular trends of ocular cancer registration in Iran. After acquiring Iranian national population-based cancer registry data, trends of age-standardised incidence rates (ASIR) of ocular cancers and annual percent changes (APC) between 2004 and 2016 were analysed in age groups, gender, topography and morphology types with joinpoint regression analysis. Age, period, and cohort effects on incidence rates were estimated by age-period-cohort model. Geographic distribution of ASIR was assessed using GIS. Overall ASIR of ocular cancers was 16.04/100,000 (95% CI 15.77-16.32). Joinpoint regression analysis showed a significant increase of ASIR between 2004 and 2009 for males (APC = 5.5, 95% CI  0.9-10.2), ages over 50 years (APC = 5.2, 1.2-9.4), skin/canthus/adnexal cancers (APC = 4.2, 0.8-7.7), and carcinomas/adenocarcinomas (APC = 4.3, 0.6-8.1); however, between 2009 and 2016 a declining trend was observed in all investigated variables. ASIR of retinoblastoma was significantly increased (averaged APC = 20.7, 9-33.7) between 2004 and 2016. age-period-cohort analyses showed that incidence rates of ocular cancers significantly increased with aging, time periods, and birth cohort effects (p < 0.001). ASIR varied from 6.7/100,000 to 21.7/100,000 in Iran. Excepting retinoblastoma, all ocular cancer incidence trends were downward over a 13-year period; however, it was increasing between 2004 and 2009 cancer. ASIR was significant aging in Iran., (© 2022. The Author(s).)

Background: Ocular surface squamous neoplasia (OSSN) is a heterogeneous group of proliferative squamous lesions on the ocular surface with varying biologic behaviours. This study aims to report the clinical profile and pathological characteristics of cases of OSSN seen at a tertiary referral centre in North West Nigeria., Methods: A retrospective review of all cases of OSSN diagnosed over a 10-year period was done., Results: OSSN accounted for 68 out of 91 ocular surface lesions affecting twice as many males as females and a peak incidence in the 30-39 years age group. They frequently presented as higher-grade and higher-stage lesions with invasive squamous cell carcinoma being the most frequently diagnosed OSSN. They also frequently showed an association with HIV infection and a relatively long duration of symptoms before presentation., Conclusion: OSSN occurs in a relatively young age group in our environment. Certain clinical and epidemiological features appear to predict the occurrence of higher-grade lesions, and this may help in the clinical prediction of likely pathologic grade and/or biologic behaviour of these lesions., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Background/aims: The primary aim of this study was to describe the causes of de novo uveitis in individuals 60 years and older. Secondary objectives were to determine the incidence of intraocular lymphoma and the clinical predictors of lymphoma., Methods: Retrospective chart review of all subjects presenting to the uveitis service at Auckland District Health Board (Auckland, New Zealand) between January 2006 and October 2020 RESULTS: 686 subjects (900 eyes) were aged ≥60 years at first presentation with uveitis, representing 23.4% of all subjects with uveitis during the study period. Non-infectious aetiology occurred in 631 (70.1%) eyes and infectious etiologies occurred in 269 (29.9%) eyes. The most frequent causes were idiopathic (36.3%), herpes zoster (14.8%), HLAB27 (8.7%) and sarcoidosis (4.8%). Twenty (2.2%) eyes of 13 (1.9%) subjects had a diagnosis of lymphoma. Lymphoma represented 11.2% of all intermediate uveitis. Subjects diagnosed with lymphoma did not develop posterior synechiae, epiretinal membrane, cystoid macular oedema or ocular hypertension., Conclusions: Intraocular lymphoma was uncommon in the overall cohort, but an important cause of intermediate uveitis. A diagnosis of lymphoma needs to be considered in any older subject with de novo intermediate uveitis. The lack of posterior synechiae, cystoid macular oedema, epiretinal membrane and ocular hypertension further increases the suspicion for lymphoma., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe the clinical features, histopathology, treatment, and outcomes of patients with ocular surface squamous neoplasia (OSSN) presenting to a referral centre in India., Methods: Retrospective interventional study., Results: Of 438 patients, the mean age at presentation was 49 years. Human immunodeficiency virus infection was noted in 72 (16%), xeroderma pigmentosum in 22 (5%), hepatitis B virus infection in 14 (3%), and systemic cancer in 8 (2%) patients. Tumor pigmentation was noted in 243 (54%) tumors with a mean percentage of tumor pigmentation of 44% (median, 40%; range, 1 to 100%). Intraocular tumor extension was noted in 12 (3%), and orbital tumor extension in 16 (4%) eyes. Of the 381 treated lesions, excisional biopsy (n = 247; 65%) was the most common treatment modality. Of the 311 lesions with histopathology diagnosis of OSSN, invasive squamous cell carcinoma (n = 92; 30%) was the most common. Over a mean follow-up period of 11 months (median, 5 months; range, 1 to 108 months) in 368 patients, tumor recurrence was noted in 16 (4%) eyes, globe salvage was achieved in 341 (90%) eyes, vision salvage in 338 (89%) eyes, regional lymph node metastasis occurred in 9 (2%), and metastasis-related death in 9 (2%) patients., Conclusion: Pigmented OSSN is common in Asian Indian population. Appropriate management of OSSN is associated with good vision, globe, and life salvage rates in India., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Oncology trends are based on data coming from different countries and ocular melanoma is the most common primary eye cancer in adults., Objectives: To investigate the incidence and characteristics of ocular melanoma in the overall population of Poland., Material and Methods: The retrospective survey of both the National Cancer Registry (NCR) and National Health Fund (NHF) databases was performed to identify all ocular melanoma cases in Poland in 2010-2017., Results: The mean incidence of ocular melanoma was 8.76/1,000,000 person-years; the lowest incidence was observed in the 19-29 age group (1.17/1,000,000 person-years) and the highest in the group over 70 (22.88/1,000,000 person-years). There were no statistically significant trends in the incidence rates over the study period. The overall incidences of uveal, eyelid and conjunctival melanoma were 6.67/1,000,000, 0.47/1,000,000 and 0.28/1,000,000 person-years, respectively. The 5-year overall survival (OS) was 60.76%; the higher risk of death was associated with male sex (hazard ratio (HR) = 1.2959), older age at diagnosis (HR = 1.0379), chemotherapy treatment (HR = 1.6774), metastasis (HR = 1.5716), loco-regional hyperplasia (HR = 1.5936), and systemic tumor spread (HR = 3.9872), compared to the carcinoma in situ. The risk of death was reduced by radiotherapy treatment (HR = 0.6645)., Conclusions: The incidence rate of ocular melanoma in Poland is in the middle of the range worldwide, and the 5-year OS is relatively low.

Background: Cumulative evidence suggests that the risk of eye tumors varies among different age groups and populations. The purpose of the present study was to assess the age distribution of eye tumors in China., Methods: In this retrospective study, the age distribution of various types of eye tumors was analyzed on surgically excised and histologically confirmed specimens obtained from 4492 patients (4526 eyes), collected between 2001 and 2017., Results: Of the 4526 specimens, 3156 eyes (69.7%) had benign eye tumors, while 1370 eyes (30.3%) had malignant tumors. The age-specific incidence of eye tumors was characterized by a bimodal distribution, one peak occurred at age 0-9 years (19.7%) and the other at 50-59 years (14.7%) of age. Malignant eyelid tumors were very rare under the age of 20 years, but increased to 78% of all eyelid tumors by the age of 70 years. Children aged 0-9 years old were 6.5 times as likely to have a malignant eye tumor (95% CI, 4.1-10.4) as those aged 10-19 years. The age-related variation of eye tumors was also observed in the top ten categories of both benign ( p < 0.001) and malignant types ( p = 0.001)., Conclusions: These results showed that age is a major factor determining the type of eye tumor, confirmed by histopathological analysis., Competing Interests: The authors declare no conflict of interest., (© 2022 The Author(s). Published by IMR Press.)

Objective: To investigate the association of high-risk hu-man papilloma virus (HR-HPV) and other risk factors with ocular surface squamous cell neoplasia (OSSN)., Materials and Methods: We obtained DNA from 22 fresh frozen OSSN tissues and 22 pterygia as controls, we used a broad-spectrum HPV DNA amplification short PCR fragment to identify HPV infection in all specimens and then genotyped HPV by a reverse hybridization line probe assay. We also obtained demographic, sun exposure, and tobacco consump-tion information., Results: HR-HPV frequency was 40.9% in the OSSN group and 4.5% in the pterygia group (p=0.009). After covariate adjustment, OSSN was associated with HR-HPV (OR=16.3, 95%CI=1.2,218.1, p=0.03) and sunburn (OR=10.8, 95%CI=1.8,86.0, p=0.02)., Conclusions: Ocular surface squamous cell neoplasia is a multifactorial disease. The strong association between HR-HPV and OSSN, suggests that HR-HPV could play an etiological role in OSSN development.

Objective: Orbito-ocular cancers are a common cause of morbidity and mortality in our population, yet these cancers have not been adequately described in Malawi. This study sought to describe the burden and histopathological profile of orbito-ocular cancers in Northern Malawi., Methods and Analysis: A retrospective review of pathology reports was done. Descriptive analyses were performed to summarise patients' demographic and clinical characteristics, and histopathological results. A binary logistic regression was used to explore the association between demographic variables, site of lesion and cancer., Results: 210 pathology reports of orbito-ocular biopsies were reviewed. 60.0% of patients were female. The majority (39.5%) of patients were in the 31 to 40 age group but overall mean age (±SD) was 34.81±15.9 years. Cancer was diagnosed in 84 (40.0%) patients. Squamous cell carcinoma constituted the majority (82.1%) of cancers followed by retinoblastoma at 7.1%. Orbito-ocular cancers were associated with HIV infection (OR5.9, 95% CI 2.0 to 17.2) and intraocular tumours were 8.3 times (OR 8.3, 95% CI 2.0 to 33.8) more likely to be malignant. However, squamous cell carcinoma was the only type of cancer found in patients with HIV infection and mostly affected the conjunctiva, constituting 94.4% of cancers affecting this site. Retinoblastoma on the other hand only affected children less than 10 years of age., Conclusion: Cancer constituted a substantial proportion of orbito-ocular lesions in our study population, with conjunctiva being the common site. This calls for improved capacity to prevent, diagnose and manage orbito-oracular cancers in Northern Malawi and similar settings., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To evaluate clinical characteristics, treatments, and outcomes in patients with ocular surface squamous neoplasia (OSSN) at a tertiary center in Northern Thailand., Methods: Patients diagnosed with either corneal-conjunctival intraepithelial neoplasia (CIN) or squamous cell carcinoma (SCC) from May 2000 to December 2015, were recruited. The patients' demographics, symptoms, clinical characteristics, cytopathology, treatments, and outcomes were reviewed., Results: Overall 171 eyes from 168 patients, 92 eyes were CIN and 79 eyes were SCC. Males were affected in 65.5%. The mean age was 58.8 ± 16.8 (29-99) years. In most cases (60.3%), the tumors were located at the limbus. The most common clinical characteristic was papilliform appearance (46.2%). Human immunodeficiency virus (HIV) infection was found in 37 (22.0%) patients with a mean age of 40.5 ± 7.7 years. The treatments and outcomes were evaluated in 136 eyes whose main initial treatment was wide excision with adjunctive cryotherapy (47.8%), followed by topical mitomycin C (30.9%). The mean follow-up time after treatment was 20.8 ± 2.2 (3-110) months and the recurrence occurred in 18 eyes (13.2%) during the follow-up period. The mean recurrence-free time (months) for CIN was significantly longer than that of SCC (81.3 ± 10.0 [95%CI 61.5 - 101.1] vs 33.2 ± 4.6 [95%CI 24.0 - 42.3], p = 0.030). SCC was the only significant risk factor that influences the recurrence of the tumors with the adjusted hazard ratio of 5.69 (p = 0.005)., Conclusion: OSSN in Northern Thailand usually involved a limbal area and presented as a papilliform mass. HIV infection should be suspected in young patients. CIN had better outcomes after treatments than invasive SCC., (© 2022. The Author(s).)

Purpose: The aim of this study was to evaluate the prevalence of histopathologically confirmed ocular surface squamous neoplasia (OSSN) in clinically diagnosed pterygium samples at a tertiary center in Northern California, over a 10-year period (2009-2019)., Methods: A retrospective chart review of patients older than 18 years with clinically diagnosed benign pterygium requiring excision was conducted. Clinically suspected pterygia were excised using standard techniques and routinely submitted to the University of California Davis for pathologic evaluation. Demographic, clinical, surgical, and pathological information were recorded and analyzed. The prevalence rate of OSSN was calculated., Results: A total of 348 consecutive specimens were evaluated. The mean (±SD) age of the patients was 58 ± 12 years, with a near equal sex representation. A total of 57 (16%) pterygia were recurrent at initial presentation. Histopathologic results demonstrated a single case of OSSN. This patient did not have a documented history of carcinoma in other organs or any history of herpes virus, human papilloma virus, or human immunodeficiency virus infection., Conclusions: The prevalence of histopathological OSSN in clinically suspected pterygia within our sample was approximately 0.3%. Because of shared clinical characteristics of pterygia and OSSN, a high index of suspicion and judicious use of anterior segment optical coherence tomography enable for effective preoperative diagnosis of OSSN. However, in the absence of clinical expertise or high-resolution optical coherence tomography, routine tissue pathologic examination may be warranted., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: The pathogenesis of ocular surface squamous neoplasia is not fully understood. Therefore, we evaluated the role of oncogenic viruses in the pathogenesis of ocular surface squamous neoplasia in Israel., Methods: Patients with ocular surface squamous neoplasia were enrolled in this retrospective study. The specimens were taken during 2004-2015 from two big centers: Emek Medical Center, Afula and the Hadassah Medical Center, Jerusalem. All the specimens (totally 26) were analyzed by immunohistochemistry for evidence of oncogenic viruses that included Human Papilloma virus, Herpes Simplex virus and Cytomegalovirus infection and 14 samples were examined by polymerase chain reaction. In addition, all the samples were examined for Epstein Barr virus infection by immunohistochemistry and Epstein Barr encoding region test., Results: Twenty-six patients were included with a mean age of 61.81 [Formula: see text] 3.83 years (mean [Formula: see text]. Immunohistochemistry staining and Epstein Barr encoding region test did not detect any of the oncogenic viruses in the 26 samples. Human Papilloma virus-16 and -18, and Herpes Simplex virus were detected by polymerase chain reaction in 14.2%, 7% and 7%, respectively., Conclusion: We conclude from our study that oncogenic viruses may play a role in the pathogenesis of ocular surface squamous neoplasia in Israel., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: To investigate the clinical features and outcomes of diffuse large B-cell lymphoma (DLBCL) involving the eye and ocular adnexa in an East Asian population and emphasize the importance of prompt biopsy and treatment in unusually progressive ocular adnexal DLBCL for better visual outcomes., Methods: Retrospective case series of 38 patients diagnosed with DLBCL involving the eye and ocular adnexa between 1995 and 2018 at a single tertiary institution., Results: There were 38 patients (22 men, 16 women), 22 (57.9%) of which had ocular adnexal lymphoma (OAL) and 16 (42.1%), vitreoretinal lymphoma (VRL). The OAL group showed higher rates of unilateral involvement (p = 0.01) and localized disease presentation (p = 0.01). Thirteen (59.1%) of 22 OAL patients were primary diseases and 9 (40.9%) were secondary. Between primary and secondary OAL groups, there was no statistical difference in clinical characteristics such as age, gender and laterality (p = 0.47, p = 0.19 and p = 0.66, respectively). All secondary OAL cases were either disseminated or relapsed by definition and only two cases (15%) of primary OAL showed disseminated presentation (p < 0.01). Six (37.3%) of 22 OAL patients showed a rapid decrease in visual acuity within 15 days and the visual recovery was irreversible in 3 of them., Conclusion: OA-DLBCL has a grave prognosis and its visual outcomes largely depend on the timing of diagnosis and treatment. When a high-grade lymphoma is suspected, prompt biopsy and treatment should be performed to ensure optimal visual, clinical and survival outcomes., (© 2021 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Objective: To identify the clinical characteristics and prevalence of neoplastic and nonneoplastic inflammatory masquerade syndromes (IMSs) in a tertiary center and determine the useful diagnostic tests., Methods: A retrospective cohort study of consecutive 1906 patients diagnosed with intraocular inflammatory disease., Results: Of all patients initially diagnosed with intraocular inflammatory disease, we identified 116 (6%) patients with noninflammatory causes (neoplastic IMSs in 36/116; 31% and nonneoplastic IMSs in 52/116; 45%). In addition, 26 patients (22%, 1.4% of all) had drug-induced uveitis and 2 (2%, 0.1% of all) had paraneoplastic uveitis. The large B-cell lymphoma was the most common neoplastic IMS (78%), and the major clinical features were presence of cells and floaters in the vitreous (69%) and chorioretinal lesions (33%). The causes of nonneoplastic IMSs included retinal vascular disorders (38%), hereditary retinal diseases (31%), and degenerative ocular disorders (19%). The common clinical manifestations consisted of chorioretinal scars (27%), small white-yellow retinal lesions (17%), and leaking vessels on fluorescein angiography (14%)., Conclusion: Noninflammatory causes were determined in 6% of a large population with initial diagnosis of intraocular inflammatory disease. Although neoplastic IMS was commonly characterized by vitreous cells and opacities, most common definitive diagnoses in nonneoplastic IMS encompassed diverse retinal disorders.

Background: Ocular surface squamous neoplasia (OSSN) is a low-grade malignancy arising from the squamous epithelium of the ocular surface AIM: To describe the clinical presentation, histological diagnoses, treatment, and outcome of treatment in patients with OSSN managed at a tertiary health facility., Methods: Medical records of all patients with OSSN managed using a standardized treatment protocol over a 10-year period were reviewed., Results: Eighty-six patients comprising 44 (51.2%) males and mean age of 48.2 ± 15.8 years were studied. The most common presentation was a fleshy growth in the eye in all patients, and 43 (50.0%) patients tested positive to human immunodeficiency virus. The right eye was affected in 44 (51.2%) patients with no bilateral tumors, and the medial limbus was involved in 28 (32.6%) patients. Morphologically, 40 (46.5%) patients had gelatinous growth, 24 (27.9%) patients were in T is category while 30 (34.9%) patients were in T 4 category. Twenty-eight (32.6%) patients received complete course of adjuvant topical mitomycin C (0.04%) while nine (10.5%) patients completed adjuvant systemic chemotherapy and external beam radiation. Overall, 61 (70.9%) patients had no tumor recurrence, seven (8.1%) patients had recurrent tumor, while the status of 18 (20.9%) patients was not known., Conclusion: OSSN occurs more commonly in younger age group in our studied population and is strongly associated with HIV seropositivity. Intraoperative cryotherapy in patients with carcinoma in situ and intraoperative cryotherapy with adjuvant topical mitomycin C in those with invasive SCC that is limited to the ocular surface are associated with low tumor recurrence., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Metastatic disease to the eye most commonly involves choroid followed by orbit leading to varied ocular manifestations. By comparison, it is relatively rarer than primary malignancies of eye as well as metastasis in other parts of the body., Aim: The aim of this study is to evaluate the common eye and orbital structures involved in secondary ocular and metastatic disease, to describe its clinical manifestations and outline the management done., Methods: A retrospective study of newly diagnosed cases of ocular metastasis in last 2 years conducted in our recently established department of ocular oncology at a tertiary eye care hospital in Nepal. Demography, age and sex distribution were noted. The patients were segregated into those with secondary or metastatic ocular malignancies. Detail study on the metastatic disease to eye was made in regards to presenting symptoms, signs, primary site of cancer, and the treatment done. Details of the investigations done, like biopsy and imaging were also recorded., Results: There were a total of 28 patients, whose age group ranged from 9 years to 69 years with median age of 43 years. Females constituted 46% of total patients. Both the eyes were involved in 9 patients (32%). Eye was secondarily involved by paranasal sinus tumors and Non Hodgkin lymphoma (7 patients each). Ocular metastasis was commonly seen from broncogenic carcinoma in four and breast carcinoma in three patients. Simultaneous metastasis to other parts of the body was also seen in 61% of our patients. Diminution of vision in 49% was the most common presenting feature followed by proptosis in 16% and palpable mass in 14% of patients. Orbit in 43% cases is the commonest ocular structure involved. Histopathologic diagnosis was done in 32% only while rest was based on imaging alone. The most common treatment done was chemotherapy in 57% patients., Conclusion: Ocular metastasis can display a wide variety of clinical and imaging features and therefore a high degree of suspicion is required. It is often associated with simultaneous metastasis to other parts of the body as well, hence the importance of earlier diagnosis and metastatic workup., (© 2021 The Authors. Cancer Reports published by Wiley Periodicals LLC.)

Purpose: To investigate the clinical features, prognostic outcomes of patients with orbital and ocular adnexal lymphoma (OALs) in Taiwanese cohort., Methods: Total 112 patients with OALs were retrospectively reviewed. Demographic information such as age, gender, lymphoma subtype, tumor location and treatment modalities were recorded. The primary endpoints were disease-specific survival (DSS), and progression-free survival (PFS)., Results: The mean patient age was 59.0 ± 15.5 years (range, 23-92 years). The major histopathologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 76 patients (67.9%), followed by diffuse large B-cell lymphoma (DLBCL) (9.8%), follicular cell lymphoma (FL) (8.0%), and small lymphocytic lymphoma (SLL) (5.4%). The anatomical locations for OALs were the orbit (44 patients, 39.3%), the conjunctiva (31 patients, 27.7%), the lacrimal gland (28 patients, 25.0%), and the eyelid (8 patients, 7.1%). With a mean follow-up time of 74.5 ± 59.8 months (range 6-342 months), the DSS for all patients were 93.1%, 87.7%, and 68.8% at 60, 120, and 180 months' follow-up, respectively. The PFS at 60, 120, and 180 months' follow-up were 86.2%, 61.2%, and 44.6%, respectively. Regarding the analysis of prognostic factors, patients with high grade lymphoma subtype and advanced stage exhibited a worse prognosis., Conclusions: MALT type lymphoma constitutes most of OALs in Taiwan and occurs more frequently than in Western countries. Patients with MALT lymphoma, FL, SLL and earlier stage have favorable outcomes compared with patients of high grade lymphoma and Ann Arbor stage IV lymphoma.

Introduction: This study was conducted to evaluate the clinical presentations and management of various Orbito-ocular malignancies in Nepal., Materials and Methods: This is a prospective study of ocular malignant cases conducted at a tertiary care eye hospital in Kathmandu, Nepal. One hundred and seventeen newly diagnosed eyes with ocular malignancy of 106 patients presenting to the clinic in a year from September 2018 to August 2019 were included for analysis. Patients' demography, clinical features, various types of malignancies seen in eyes and their management were evaluated., Results: The mean age of patients was 30.35 years with a range from 1 month to 84 years and standard deviation of 26.63. 56% of the total were male. Red eyes and visible mass were the most common symptoms in 23% with intraocular contents in 48% being the most common site involved. Different types of ocular malignancies were detected, among which retinoblastoma was the most common in 38% of patients. Diagnosis in most patients was clinical and/or radiological; incision biopsy was required in 1.7% patients only. Surgical excision of the tumor was the most common treatment done in 29% followed by enucleation in 27% of eyes. Bilateral involvement of the eyes was seen in 10% of the patients., Conclusion: Malignant orbito-ocular tumors can be seen in any age group. Retinoblastoma is the commonest malignancy seen. With the help of imaging, diagnosis is near accurate; hence biopsy is not required in most cases before commencing treatment., (© NEPjOPH.)

Background: Nationwide studies of ocular adnexal lymphoma (OAL) are very rare in the literature, and knowledge on incidence, subtype distribution and long-term survival data is limited. This is the largest national study of OAL to date. This study sought to find information on incidence, changes in incidence, clinical findings, distribution of subtypes, survival and prognostic factors., Methods: Patients diagnosed with OAL from January 1, 1980 to December 31, 2017 were identified in Danish registers, and clinical as well as survival data were collected. The data were analysed with Kaplan-Meier plots and log-rank test., Results: 387 patients were included in the study. The major lymphoma subtypes were extranodal marginal-zone B cell lymphoma (EMZL) (55%), diffuse large B cell lymphoma (DLBCL) (13%), mantle cell lymphoma (MCL) (11%) and follicular lymphoma (FL) (10%). OAL is a disease of the elderly (median age 69 years). The incidence of lymphoma of the ocular adnexal region has increased significantly throughout the time period of the study (Pearson correlation coefficient, r=0.65; P<0.001). In the period 1980-1984, the incidence was 0.086 per 100 000, which increased to 0.307 per 100 000 in the period 2013-2017. Low-grade, low-stage primary lymphomas were treated with radiotherapy, whereas patients with high-stage, high-grade and/or relapsed disease were treated with chemotherapy. Low-grade subtypes EMZL (89%) and FL (56%) had better 10-year disease-specific survival than the high-grade lymphomas DLBCL (38%) and MCL (31%)(p<0.001)., Conclusion: OAL is increasing in incidence in the Danish population for unknown reasons. However, the prognosis for most OAL is favourable, as highlighted in this national long-term study., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose : To perform a literature review on the primary malignant epithelial tumors (PMET) involving the lacrimal drainage system (LDS). Methods : The authors performed a PubMed search of all articles published in English on primary malignant epithelial tumors of the lacrimal sac and the nasolacrimal duct. Data analyzed include prevalence, demographics, clinical presentation, histopathological subtypes, management modalities, outcomes, and recurrence rates. Results : Malignant epithelial tumors of the lacrimal sac are commonly diagnosed in the fifth to seventh decades of life (mean age: 58 years) and have no gender predilection. Typical clinical presentation includes epiphora (84%) and lacrimal fossa mass lesion (78%). Pain (9%) and blood-stained epiphora (4%) were inconsistent findings. Among the PMET, squamous cell carcinomas (SCCs) were the most common (61%) followed by transitional cell carcinomas (TCC; 15%), and mucoepidermoid carcinoma (MEC; 7%). Complete surgical excision followed by radiotherapy (59%) was the preferred modality of management, and only 18% required orbital exenteration. Of the 331 tumors with available treatment details, 85% survived at a mean follow-up of 33 months. The overall recurrence rate following treatment was 20% (65/331). MECs of the lacrimal sac had the best while adenoid cystic carcinoma had the worst prognosis. Conclusion : Blood-stained epiphora was not the classical presentation for primary epithelial malignancies of the LDS. No validated staging system exists for lacrimal drainage tumors. There is a need for consistent and detailed reporting in the literature, more so on the histological subtypes and their management.

Purpose: Xeroderma pigmentosum (XP) is an extreme hypersensitivity to sunlight causing skin freckling and pigmentary changes because of defective DNA repair mechanisms. The purpose of this article is to evaluate the spectrum of ocular and systemic features in XP at a tertiary eye care center in India over 32 years., Methods: Data from 418 eyes of 209 patients diagnosed with XP from 1987 to 2018 were reviewed retrospectively for demographics, complaints, ocular features, systemic associations, and their management., Results: Median age at diagnosis was 2 years (interquartile range, 0.5-5 years). A total of 124 patients (59.3%) were men. There was parental consanguinity in 74.4% cases. Common ocular complaints were photophobia (47.1%), ocular discomfort (45%), defective vision (36.6%), redness (13.4%), tissue growth (12%), white spot (11.2%), and pain (10.5%). At presentation, 43.5% had corneal scars (45.5% were located inferiorly and 70.9% covered visual axis). Corneal vascularization and limbal stem cell deficiency were noted in 37.4%. A total of 56% of patients had at least 1 tumor, and 6.7% had neurological abnormalities. At least 1 ocular surgery was performed in 37.8% of patients. Ophthalmic surgical interventions included tumor excision (23%), keratoplasty (13.4%), and nontumor ocular surface surgery (3.4%)., Conclusions: XP is a disorder that has high ocular morbidity in Indian patients. The recognition of common signs and symptoms and relative frequency of various ocular complications with time trends will help in managing and reducing the sequelae of this otherwise untreatable and progressive disease., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Although studies have investigated the risk of second primary malignancies (SPMs) associated with lymphoma of various sites, limited studies have investigated this risk in patients with lymphoma originating within the ocular adnexa. We conducted a retrospective study to assess incidence of secondary malignancies in patients with a prior diagnosis of ocular adnexal lymphoma (OAL) and to determine latency periods and age-groups at increased risk for SPM occurrence., Methods: Retrospective analysis was performed on data obtained from Surveillance, Epidemiology, and End Results (SEER) 9 database. Patients with an initial primary malignancy diagnosis of OAL between 1973 and 2015 were included in the study. Standardized incidence ratios (SIR) and excess absolute risks (EAR) compared to a SEER reference population with similar sex, race, age, and calendar year were computed for SPMs. Excess absolute risk is per 10,000 individuals; alpha of 0.05 was used., Results: Of 1834 patients with primary ocular adnexal lymphoma, 279 developed a secondary malignancy during average follow-up of 110.03 months (+/- 88.46), denoting higher incidence than expected (SIR 1.20; 95% CI, 1.07 to 1.35; EAR 30.56). Amongst the primary lymphoma cohort, 98.7% (1810/1834) of patients had non-Hodgkin's lymphoma and amongst those that developed secondary malignancies, 99.6% (278/279) had non-Hodgkin's lymphoma. Patients exhibited increased incidence of lymphohematopoietic and non-lymphohematopoietic second malignancies and no secondary malignancies of the eye or orbit. Patients had increased incidence of secondary malignancies in the first year (SIR 2.07; 95% CI, 1.49 to 2.79; EAR 150.37) and 1-5 years following lymphoma diagnosis (SIR 1.24; 95% CI, 1.01 to 1.51; EAR 34.89). Patients with various OAL subtypes demonstrated differing patterns of site-specific and overall SPM risk., Conclusions: Patients with prior diagnosis of ocular adnexal lymphoma possess increased risk of hematologic and non-hematologic secondary malignancies. Risk of secondary malignancy could vary by lymphoma subtype. Patients with ocular adnexal lymphoma may benefit from regular surveillance to promote early detection of second primary malignancies.

Introduction: Limited data are available on the incidence of primary ophthalmic cancers worldwide. We describe the incidence and trends of primary ophthalmic cancers in Singapore., Methods: Data on ophthalmic cancers diagnosed in Singapore from 1996 to 2016 were retrieved from the Singapore Cancer Registry for analysis. All were histologically proven primary ophthalmic cancers. Calculations of incidence and age-specific frequency of ophthalmic malignancy were made., Results: A total of 297 cases were included, with males constituting 59.9%. The race distribution was 78.5% Chinese, 16.5% Malay, 3.7% Indians and 1.3% others. There was an overall increase in ophthalmic malignancies. The mean age of onset was 47.4 years. The most common cancers were retinoblastoma (93.3%) in patients younger than 15 years, and lymphoma (71.3%) in patients aged 15 years and older. There has been an increase in lymphomas from 16.7% in 1968-1995 to 71.3% in 1996-2016 in those aged 15 years and older. The most common types of ophthalmic cancer according to location are lymphoma of the orbit, conjunctiva, cornea and lacrimal gland; retinoblastoma of the retina; and malignant melanoma of the choroid and ciliary body., Conclusion: Our study reported the incidence and trends of ophthalmic cancer in the Singapore population and showed an overall increase in ophthalmic malignancies in Singapore from 1996-2016. A substantial increase in lymphomas over the last 2 decades was noted. The data could aid clinicians, epidemiologists and policymakers in implementing strategies to address trends in ophthalmic cancers and spur aetiological research to improve quality of life in patients with such cancers.

The COVID-19 pandemic has had an unprecedented impact on the National Health Service in United Kingdom. The UK Ocular Oncology Services evaluated the impact on the adult eye cancer care in the UK. All four adult Ocular Oncology centres participated in a multicentre retrospective review comparing uveal melanoma referral patterns and treatments in a 4-month period during the national lockdown and first wave of the COVID-19 pandemic in 2020 with corresponding periods in previous 2 years. During the national lockdown, referral numbers and confirmed uveal melanoma cases reduced considerably, equalling to ~120 fewer diagnosed uveal melanoma cases compared to previous 2 years. Contrary to the recent trend, increased caseloads of enucleation and stereotactic radiosurgery (p > 0.05), in comparison to fewer proton beam therapy (p < 0.05), were performed. In the 4-month period following lockdown, there was a surge in clinical activities with more advanced diseases (p < 0.05) presenting to the services. As the COVID-19 pandemic continues to mount pressure and reveal its hidden impact on the eye cancer care, it is imperative for the Ocular Oncology Services to plan recovery strategies and innovative ways of working.

Purpose: The eye and its adnexal structures can give rise to first or consecutive primary malignancies or to encounter metastasis. Our aim was to define the characteristics of the second primary neoplasms affecting the eye and its adnexa and find the risk modifying factors for them after malignancies elsewhere in the body., Methods: We have queried the Surveillance, Epidemiology and End-Results "SEER"-9 program of the National Cancer Institute for the malignancies of the eye and its adnexa that occurred between 1973 and 2015. The malignancies were ordered chronologically according to their incidence: first or second primary malignancies. The tumors were classified according to ICD-O-3 classification. Standardized incidence ratios (SIR) and survival probabilities were calculated for subgroups., Results: Among 3,578,950 cancer patients, 1203 experienced a second malignancies of the eye and its adnexa. The first malignancy was diagnosed between 50 and 69 years of age in 58.94% of them. The eyelid showed 280 events, while 50 in lacrimal gland, 181 in the orbit, 21 in the overlapping lesions, 15 in optic nerve, 148 in the conjunctiva, 9 in the cornea, 6 in the Retina, 379 in the choroid, and 93 in the ciliary body. The SIR of a second malignancy after a prior non-Hodgkin lymphoma was 2.42, and in case of previous skin carcinomas it was 3.02, melanoma of skin, and 2.13 and 1.58 in oral cavity/pharynx malignancies. The second ocular and adnexal neoplasms increased steadily over the 5-year periods on contrary to first primary neoplasms. The survival of patients affected with first ocular and adnexal neoplasms was significantly higher than those with second ocular and adnexal neoplasms. On the other side, second primary ocular and adnexal tumors showed a better survival than second primary malignancies elsewhere., Conclusions: The epidemiological differences between first and second ocular and adnexal primaries suggest different underlying mechanisms. Careful ocular examination should be integrated in the long-term follow-up plan of cancer patients. Special attention should be given to patients with non-Hodgkin's lymphoma and melanoma as first primary.

Invasive ductal carcinoma (IDC) is a major type of breast cancer. Ocular metastasis (OM) in IDC is rarely seen, but patients with OM often have a poor prognosis. Furthermore, OM is difficult to detect in the early stages by common imaging examinations. In the present study, we tried to figure out the risk factors of OM in IDC and evaluate their diagnostic values for early detection. There were 1192 IDC patients who were divided into two groups according to ocular metastasis involved in this study. Clinical parameters of those patients were used to detect differences. The binary logistic regression test was then used to determine the risk factors of OM in IDC. Furthermore, ROC curves of both single and combined risk factors were established to examine their diagnostic values. The incidence of axillary lymph node metastases was significantly higher in the OM group (p = 0.002). Higher carbohydrate antigen 153 (CA153), lower apolipoprotein A1 (ApoA1), and hemoglobin (Hb) were risk factors for OM in IDC (p < 0.001, p < 0.001, p = 0.038, respectively). In the single risk factor ROC analysis, cutoff values of CA153, ApoA1, and Hb were 43.3 u/mL (CI: 0.966-0.984, p < 0.001), 1.11 g/L (CI: 0.923-0.951, p < 0.001), and 112 g/L (CI: 0.815-0.857, p < 0.001), respectively. Among the ROC curves of combined risk factors, CA153+ApoA1+Hb had the best accuracy, with the sensitivity and specificity of 89.47% and 99.32%, respectively (CI: 0.964-0.983, p < 0.001). CA153, ApoA1, and Hb are risk factors for OM in IDC. In clinical practice, the three parameters could be used as predictive factors for the early detection of OM., (© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Purpose: To describe the epidemiology of ocular and periocular tumors in patients presenting to a multi-tier ophthalmology hospital network in India using the electronic medical records (EMRs) system., Methods: A retrospective, observational, referral, hospital-based study of 1,142,098 patients. The data were collected using the in-house developed EyeSmart EMR system., Results: During a 6-year study period, 9633 (0.8%) new patients were diagnosed with eye tumors. Of the 9633 patients, 5209 (54%) were male and 4424 (46%) were female. Of all tumors, 6372 (65%) were benign, 282 (3%) were pre-malignant, and 3089 (32%) were malignant in nature, respectively. Overall, the three most common tumors included retinoblastoma ( n = 1167, 12%), ocular surface squamous neoplasia ( n = 957, 10%), and conjunctival nevus ( n = 903, 9%). The three most common benign tumors included conjunctival nevus ( n = 903, 9%), eyelid nevus ( n = 358, 4%), and orbital dermoid cyst ( n = 344, 4%). The three most common malignant tumors included retinoblastoma ( n = 1167, 12%), ocular surface squamous neoplasia ( n = 957, 10%), and sebaceous gland carcinoma ( n = 202, 2%). The most common tumor in 0-10 years' age group was retinoblastoma ( n = 1163, 42%), 11-30 years was conjunctival nevus ( n = 408, 16%), and > 30 years was ocular surface squamous neoplasia ( n = 801, 17%), respectively., Conclusions: The present study results indicate the incidence and distribution of ocular and periocular tumors in a large cohort in India. Retinoblastoma is the most common tumor encountered in a referral-based comprehensive ophthalmic oncology practice in India. The use of EMRs enables to capture the structured information and big data analysis of the same., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Middle East African Journal of Ophthalmology.)

Background: The outcomes of patients with postmenopausal breast cancer(PBC) can be improved through the early detection of intraocular metastases(IOMs). In this study, we investigated patients with PBC, and compared those with IOMs with those with non-intraocular metastases(NIOMs) in terms of blood lipid levels, and then differentiated the risk factors associated with IOMs., Methods: Student's t-test and a chi-square test were used to discriminate between the IOMs and NIOMs groups. After establishing a Poisson regression model to analyze risk factors, we plotted receiver operating characteristic curves(ROC) to assess the quality of risk factors predicting IOMs., Results: The incidence of IOMs in PBC was 1.16%. There was no significant difference in terms of histopathology between the 2 groups. The levels of total cholesterol (TC), apolipoprotein A1(APOA1) and low-density lipoprotein(LDL) in IOMs were significantly lower than in NIOMs groups. Poisson regression suggested that low levels of APOA1 and LDL were risk factors for IOMs (P = 0.002 and P < 0.001, respectively). ROC curve analysis demonstrated that the cut-off values of APOA1 and LDL were 1.025 g/L and 2.415 mmol/L. The highest prediction accuracy for IOMs involved the combination of APOA1 and LDL (AUC = 0.881, P < 0.001)., Conclusion: Our research demonstrates that low levels of APOA1 and LDL efficiently predict IOMs in PBC as risk factors, and the combination of APOA1 and LDL was more predictive than single factors.

Nasopharyngeal carcinoma (NPC) has a distinctive geographical distribution in China, especially southern China. There are several risk factors for NPC, such as Epstein-Barr virus, genetics, and environmental exposures. Although the incidence of eye metastasis (EM) is lower than metastasis in other body parts, it often indicates poor prognosis.We assessed several serum biomarkers for their ability to predict EM in NPC. Patients with NPC were selected (n = 963), and were separated into two groups, EM and no eye metastasis. Ten factors were analyzed in both groups including triglyceride (TG), high-density lipoprotein, low-density lipoprotein, alkaline phosphatase, alpha fetoprotein, carbohydrate antigen-199, cancer antigen-153, apolipoproteins AI, apolipoprotein B, and cytokeratin fragment 19 (CYFRA21-1). Independent t tests, binary logistic regression, and receiver operating characteristic curves were used to assess the data.The EM group had significantly higher CYFRA21-1 and lower TG compared with the no eye metastasis group. Areas under the curve for CYFRA21-1, TG and CYFRA21-1/TG were 0.966, 0.771, and 0.976, respectively. The corresponding cut-off values were 12.12 ng/ml, 0.41 mmol/L, and 13.5. The sensitivity and specificity of CYFRA21-1/TG were 100% and 92.2%, respectively.The increased ratio of CYFRA21-1 to TG can be an accurate method to detect EM in patients with NPC.

Purpose: To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival., Design: Multicenter retrospective interventional case series., Methods: Patient data were collected from 6 international eye cancer centers from January 1, 1980, through December 31, 2017. All patients with histologically verified primary or secondary lymphoma of the lacrimal gland were included. Primary endpoints were overall survival (OS) and disease-specific survival (DSS)., Results: A total of 260 patients with lacrimal gland lymphoma were identified. The median age was 58 years and 52% of patients were men. Non-Hodgkin B-cell lymphomas constituted 99% (n = 258) and T-cell lymphomas constituted 1% (n = 2). The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n = 177, 68%), follicular lymphoma (FL) (n = 26, 10%), diffuse large B-cell lymphoma (DLBCL) (n = 25, 10%), and mantle cell lymphoma (MCL) (n = 17, 7%). Low-grade lymphomas (EMZL and FL) were most commonly treated with external beam radiotherapy (EBRT), whereas high-grade lymphomas (DLBCL and MCL) were treated with chemotherapy in combination with rituximab and/or EBRT. The prognosis was relatively good with a 5-year OS and DSS of 73.8% and 87.5%, respectively. Lymphoma subtype was a statistically significant predictor for DSS, with EMZL (5-year DSS: 93.4%) having the best prognosis and DLBCL (5-year DSS: 52.6%) having the poorest., Conclusions: This is the largest reported collection of data of subtype-specific lacrimal gland lymphoma. The subtype distribution of lacrimal gland lymphoma resembles that of the ocular adnexa. Prognosis is good and the histologic subtype is a significant predictor for disease-specific survival., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Helicobacter pylori has been proposed as a possible etiologic factor of ocular adnexa lymphoma (OAL), although with conflicting results. To assess the involvement of H. pylori in OAL, as (1) H. pylori DNA positivity on OAL specimens, and (2) prevalence of H. pylori gastric infection in patients with OAL. A systematic review of studies assessing H. pylori in patients with OAL was conducted by searching electronic databases from their inception to May 2019. Pooled positivity for H. pylori in OAL specimens detected by polymerase chain reaction, and pooled prevalence of H. pylori gastric infection, were calculated with 95% confidence interval (CI). Eleven studies with 308 patients were included. Pooled positivity for H. pylori was 16.8% in all OALs and 22.7% in MALT OAL, with high heterogeneity among studies. Pooled prevalence of H. pylori gastric infection in patients with OAL was 34.7%, with low statistical heterogeneity. In conclusion, H. pylori seems to be involved in a subset of OAL, but the heterogeneity found needs to be investigated in further studies. The prevalence of H. pylori gastric infection in patients with OAL does not seem to differ from that of the general population.