Treatment of ocular surface squamous neoplasia in an Indian rural facility: a study of 38 eyes.

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.

Clinical features and prognosis of conjunctival melanoma in Japanese patients.

PURPOSE: To evaluate the clinical features and prognosis of conjunctival melanoma in Japanese patients. STUDY DESIGN: Retrospective observational case series. METHODS: Twenty patients (8 men and 12 women) diagnosed with conjunctival melanoma at a singlehospital between 2003 and 2017 were analyzed. Data on clinical presentation, sex, age, the affected eye, tumor location, tumor origin, tumor stage according to the American Joint Committee on Cancer staging system (eighth edition), treatment, outcomes, local recurrence, metastasis, and survival were extracted from the patients' medical records and reviewed. RESULTS: The mean age at diagnosis was 64.2 ± 14.8 years. Tumor locations at the first examination included the bulbar conjunctiva (n = 19), plica (n = 13), and fornix (n = 12). The tumor stage was T1 in 5 cases (25%), T2 in 12 cases (60%), T3 in 3 cases (15%), and T4 in none. The mean follow-up duration was 91.7 ± 46.0 months. The local recurrence rates at 1, 5, and 10 years were 5.0%, 18.8%, and 31.5%, respectively, whilst the metastasis rates were 5.0%, 25.6%, and 32.4%, respectively. Four of the 6 patients who experienced metastasis died; duration from metastasis to death was 17.5 months (range, 7-25). The 5-year survival rate for conjunctival melanoma was 78.8%. Tumor thickness was significantly associated with survival duration on univariate Cox regression analyses. CONCLUSION: The mortality rate for conjunctival melanoma in the Japanese population was lower and higher than that reported in the Chinese and United States populations, respectively. Tumor thickness was a prognostic factor for survival in patients with conjunctival melanoma.

Ambient ultraviolet radiation and ocular melanoma incidence in the United States, 2000-2019.

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/m2), 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.

Incidence of ocular surface squamous neoplasia in pterygium specimens.

OBJECTIVE: Pterygium and ocular surface squamous neoplasia (OSSN) have been recognized as likely related conditions and share similar risk factors such as ultraviolet radiation and chronic inflammation. The purpose of this study is to review the incidence of OSSN in pathology specimens sent as pterygium at a single tertiary centre between 2010 and 2022. METHODS: This is a retrospective chart review of patients operated on for pterygium between 2010 and 2022 at the University of Montreal Health Centre. Data collected include baseline demographics, results of pathology specimen, and clinical information for cases diagnosed as OSSN on pathology. RESULTS: A total of 1559 patients were operated on for a clinical diagnosis of pterygium between 2010 and 2022, of which 854 patients (55%) were male. A total of 1142 specimens had available pathology reports, and most of the specimens were consistent with pterygium on pathology (1105 of 1142; 97%). There was an unexpected finding of 3 cases of OSSN (3 of 1142; 0.3%). Other diagnosis besides pterygium were seen in 3% of specimens (34 of 1142), including nevus (n = 12), spheroidal degeneration (n = 3), pyogenic granuloma (n = 3), and lymphangiectasia (n = 2). The 3 cases of OSSN included an 81-year-old male of French-Canadian background, a 52-year-old male of South Asian background, and a 59-year-old female of French-Canadian background. The pathology was diagnosed as conjunctival intraepithelial neoplasia (CIN) grade 3, CIN grade 2, and CIN grade 2, respectively. CONCLUSION: The finding of OSSN in pterygium is rare in our population but can be clinically difficult to distinguish. It is important to send all pterygium specimens for pathology.

Incidence and Mortality of Conjunctival Melanoma in Australia (1982 to 2014).

Purpose: The purpose of this study was to estimate the incidence and mortality of conjunctival melanoma in Australia from 1982 to 2014. Methods: De-identified unit data for all cases of ocular melanoma were extracted from the Australian Cancer Database from 1982 to 2014. Conjunctival melanoma cases were extracted, and the incidence and mortality were analyzed. Incidence rates were age-standardized against the 2001 Australian Standard Population. Mortality was assessed using log-rank and Cox regression. Results: From 1982 to 2014, there were 299 cases of conjunctival melanoma. The age-standardized incidence rate was 0.48 (95% confidence interval [CI] = 0.41 to 0.54) per million per year. Women (0.52, 95% CI = 0.42 to 0.62) had a higher incidence than men (0.42, 95% CI = 0.33 to 0.51). The incidence of conjunctival melanoma increased in men (+1.46%) and significantly women (+1.41%, P = 0.023) over the study period. The mean 5-, 10-, and 15-year disease-specific survival were 90%, 82%, and 80%, respectively, during the 33-year interval. Comparisons of survival among age, sex, and state revealed no significant differences when tested using log-rank or Cox regression. Conclusions: In conclusion, we found an increase in the rate of conjunctival melanoma diagnoses in Australia from 1982 to 2014. Over the same period, disease survival remained unchanged at a mean of 90%.

Conjunctival Involvement in Infants as an Unusual Symptom of Omicron XBB.1.16 Driven Surge.

We describe clinical characteristics of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infected children during the XBB.1.16 variant-driven surge in April, 2023 in India. A signi-ficantly higher positivity rate in young infants than in older children (37.4% vs 13.3%; P<0.001), and a predominance of respiratory symptoms were noticed. Notably, non-purulent con-junctivitis was found in 36.8% of SARS-CoV-2 positive infants. All recovered with symptomatic treatment as outpatients.

Histopathological Results of Conjunctival Masses with Suspected Malignancy Based on Slit-lamp Biomicroscopy.

OBJECTIVE: To evaluate the histopathological results of conjunctival masses suspected to be malignant based on biomicroscopic examination. STUDY DESIGN: Observational study. Place and Duration of the Study: Department of Ophthalmology, Celal Bayar University, Hafsa Sultan Hospital, Manisa, Turkiye, from November 2013 to February 2021. METHODOLOGY: Patients who underwent excision for the diagnosis and treatment of conjunctival surface masses with clinical suspicion of malignancy were evaluated retrospectively. The masses were categorised as benign, premalignant, and malignant lesions according to the histopathological results. The distribution of conjunctival masses was analysed according to patients' gender and age groups. RESULTS: The study included 98 conjunctival masses in 98 patients (57.1% men, mean age 48.6±21.85 years). On pathological examination, 63 of the masses were found to be benign (64.3%), 22 were premalignant (22.4%), and 13 were malignant (13.3%). The most common benign lesion was nevus (n=21, 21.4%). All detected premalignant lesions were conjunctival intraepithelial neoplasms (CIN), detected in 22 patients (22.4%). Of these, 13 patients (13.3%) had carcinoma in situ (CIN III). Thirteen (13.3%) of the masses were malignant, the most common being squamous cell carcinoma (n=10, 10.2%). The frequency of premalignant and malignant lesions increased with age (p<0.001). There was no statistical relationship between malignancy and gender (p=0.619). CONCLUSION: Most conjunctival masses in this series were benign, with nevi being the most common. The most common premalignant and malignant lesions were intraepithelial neoplasia and squamous cell carcinoma, respectively. Malignant conjunctival lesions were more common in older patients. KEY WORDS: Conjunctival nevus, Conjunctival intraepithelial neoplasia, Squamous cell carcinoma.

Decreasing incidence of conjunctival squamous cell carcinoma in people with HIV in South Africa.

BACKGROUND: The main risk factors for squamous cell carcinoma of the conjunctiva (SCCC) are immunodeficiency and exposure to ultraviolet radiation. Little is known about SCCC epidemiology among people with HIV (PWH) in South Africa. METHODS: We used data from the South African HIV Cancer Match study, a nation-wide cohort of PWH in South Africa, created through a privacy-preserving probabilistic record linkage of HIV-related laboratory records from the National Health Laboratory Service and cancer records from the National Cancer Registry from 2004 to 2014. We calculated crude incidence rates, analyzed trends using joinpoint models, and estimated hazard ratios for different risk factors using Royston-Parmar flexible parametric survival models. RESULTS: Among 5 247 968 PWH, 1059 cases of incident SCCC were diagnosed, for a crude overall SCCC incidence rate of 6.8 per 100 000 person-years. The SCCC incidence rate decreased between 2004 and 2014, with an annual percentage change of ‒10.9% (95% confidence interval: ‒13.3 to ‒8.3). PWH residing within latitudes 30°S to 34°S had a 49% lower SCCC risk than those residing at less than 25°S latitude (adjusted hazard ratio = 0.67; 95% confidence interval: 0.55 to 0.82). Other risk factors for SCCC were lower CD4 counts and middle age. There was no evidence for an association of sex or settlement type with SCCC risk. CONCLUSIONS: An increased risk of developing SCCC was associated with lower CD4 counts and residence closer to the equator, indicative of higher ultraviolet radiation exposure. Clinicians and PWH should be educated on known SCCC preventive measures, such as maintaining high CD4 counts and protection from ultraviolet radiation through sunglasses and sunhats when outdoors.

Demographics, clinical presentation and risk factors of ocular surface squamous neoplasia at a tertiary hospital, South Africa.

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.

Conjunctival Melanoma in Aotearoa-New Zealand: A 21-Year Analysis of Incidence and Survival.

PURPOSE: Conjunctival melanoma (CM) is a rare and aggressive malignancy. Global studies demonstrate increased burden of disease in countries with high rates of cutaneous melanoma. There are currently no reports on CM incidence, trends, or survival within Aotearoa-New Zealand (NZ), a country with the highest global rates of cutaneous melanoma, which this study aims to address. DESIGN: This was a retrospective review using the national cancer registry. METHODS: Data on histologically confirmed CM diagnosed between January 1, 2000, and December 31, 2020, were obtained from the NZ Cancer Registry. Cases were identified using the International Classification of Disease, 10th edition (ICD-10) codes. Primary outcome measures were age-standardized incidence, trends, and survival. RESULTS: A total of 68 CM cases were identified. There was a preponderance for females (n=40, 58.8%) and CM predominantly affected European patients (n=63, 92.6%). Median follow-up was 5.0 years [interquartile range (IQR)=2.4-9.9 y] and the median age at diagnosis was 68.5 years (IQR=57.0-79.0 y), with non-Europeans presenting at a significantly younger age [-17.3 y (95% CI: -31.3 to -3.2), P =0.019] than Europeans. The annual age-adjusted incidence(±SD) was 0.6±0.2 cases per million population per year with a stable incidence trend over 21 years. All-cause mortality was found in 28 cases (41.2%) and the median time to death was 3.76 years (IQR=2.1-5.7 y). Five-year all-cause survival and disease-specific survival was 69% and 90%, respectively. CONCLUSIONS: This is the first report on CM incidence, trends, and mortality in NZ. The CM burden is in line with European and North American data, despite NZ having the highest rate of cutaneous melanoma. The incidence remained stable over 2 decades.

Trends in Incidence of Conjunctival Melanoma in the US.

This cohort study assesses the incidence of conjunctival melanoma, associations between demographic factors, and trends over time in the US.

Association of high-risk human papillomavirus with ocular surface squamous neoplasia: a case-control study in Mexico.

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.

Ocular surface squamous neoplasia in Northern Thailand: a 16-year review.

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.

Ocular surface squamous neoplasia in India: a study of 438 patients.

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.

Demographic and clinical features of conjunctival tumours at a tertiary care centre.

CLINICAL RELEVANCE: This study investigates the demographic and clinical features of conjunctival tumours. BACKGROUND: Conjunctival tumours include a large spectrum of conditions ranging from benign lesions to aggressive, life-threatening malignancies. Knowing the distribution of conjunctival tumours by age and gender is important for reducing cancer morbidity. METHODS: The clinical records of 375 patients (410 eyes) diagnosed with a conjunctival mass at a tertiary referral centre between February 1999 and November 2020 were retrospectively evaluated. RESULTS: Two-hundred-seventeen (57.9%) patients were male and 158 (42.1%) were female. Of 410 conjunctival tumours, 159 (38.8%) were benign, 106 (25.9%) premalignant, and 145 (35.4%) malignant. Overall, the 3 most common diagnoses were squamous cell carcinoma (SCC, 19.5%), conjunctival intraepithelial neoplasia (CIN, 18.3%), and naevus (17.8%). The most common benign, premalignant, and malignant tumours were naevus (n = 73/159, 45.9%), CIN (n = 75/106, 70.8%), and SCC (n = 80/145, 55.2%) respectively. Naevus was the most common tumour in ≤20 years and > 20-40 years old patient groups (56.2% and 25.4% respectively). CIN was the most frequent tumour in patients aged > 40-60 years (25.7%). SCC was the most common tumour in > 60-80 years and > 80 years old patient groups (44.3% and 80.0% respectively). The median patient age was greater in patients with malignant tumours (64.5 years) compared to patients with premalignant (55.5 years, p = 0.011) and benign tumours (22.0 years, p < 0.001). Malignant tumours displayed larger base diameter, greater thickness, and intrinsic vessels compared to premalignant or benign lesions (p < 0.001 for each parameter). Malignant tumours also displayed more amelanotic vs melanotic appearance (p < 0.001) and limbal vs extralimbal bulbar location compared to benign lesions (p < 0.001). CONCLUSION: Premalignant and malignant tumours comprised 61.2% of all conjunctival tumours and were usually detected in patients > 40 years of age in this study.

Conjunctival Lesions: A 5-Year Basic Demographic Data and Clinicopathological Review in a Tertiary Eye Care Hospital.

BACKGROUND: Conjunctival lesions are common with a wide spectrum of benign, premalignant, and malignant lesions. Few histopathological studies have been conducted on conjunctival lesions with variable designs and results. Our aim in this study is to provide information on common conjunctival lesions seen in an ophthalmology tertiary care center in Saudi Arabia. METHODS: A retrospective, observational study of all consecutive conjunctival tissue specimens sent for histopathological assessment to the pathology department from 2015 to 2019 were analyzed. Clinical data were collected from medical records, and the histopathological slides were reviewed by a single pathologist. RESULTS: A total of 110 conjunctival specimens from 108 patients were included (mean age: 53 years, 67 males and 43 females). Bilateral involvement was mostly found in inflammatory lesions (40%). Most lesions were benign (91%), with a significantly longer duration of symptoms in malignant lesions (p = 0.036*). The clinical diagnosis matched the final histopathological diagnosis in 75.5% of the total specimens. The most frequent category of benign lesions was fibrodegenerative and proliferative lesions (53.6%), with a significantly higher prevalence among adult males (p < 0.001). Melanocytic lesions were more common in children (33.3%) than adults (9.8%), and the mean age of children was significantly lower (p = 0.013). The most frequent malignant lesion was ocular surface squamous neoplasia (50%), with equal prevalence among males and females. The overall outcome was favorable in 89.4% and unfavorable in 10.6%, mostly due to surgical complications, further progression of the lesion, or recurrence. CONCLUSION: This study shows variability in the frequency of conjunctival lesions based on gender, age, geographical, racial, and environmental factors. There has been a shift in the gender-based prevalence of ocular squamous neoplasia over the last three decades, probably due to a change in lifestyle.

Ocular surface squamous neoplasia in New Zealand: a ten-year review of incidence in the Waikato region.

BACKGROUND: Ocular surface squamous neoplasia (OSSN) is a relatively rare disease with a low mortality and highly variable incidence. Despite a high incidence of OSSN in the Southern hemisphere, there is limited epidemiological data for New Zealand. The current study aims to assess the incidence, demographics, and histological grade of OSSN in the Waikato region of New Zealand, home to ~10% of the population of New Zealand. METHODS: Non-interventional retrospective cohort study. All conjunctival biopsy histology reports from 2010 to 2019 in the Waikato region of New Zealand were analysed. Age, sex, and ethnicity were analysed and the incidence of OSSN was calculated. Main outcome measures included histological grade, rate of recurrence, and incidence of OSSN. RESULTS: A total of 386 patients underwent conjunctival biopsy with histology during the study period. Eighty-three lesions (22%) involving 80 patients (21%) were reported positive for OSSN. Patients with OSSN had a mean age of 68.9 years (SD = 13.2), were predominantly male (76%), and of New Zealand-European ethnicity (53%). Conjunctival intraepithelial neoplasia-1 (30%) was the most frequent diagnosis. Three patients (4%) had recurrent lesions requiring repeat biopsy. The peak annual OSSN incidence rate was 3.81/100,000 population in 2019. The overall ten-year incidence was 2.13/100,000 population/year. CONCLUSION: This is the largest study to investigate OSSN incidence in New Zealand. The incidence rate of OSSN is one of the highest rates reported in the literature.

Second Primary Malignancies following a Diagnosis of Conjunctival Melanoma.

BACKGROUND: Although incidence of second primary malignancies (SPMs) has been investigated in patients with cutaneous melanoma and uveal melanoma, limited studies have investigated their occurrence subsequent to conjunctival melanoma (CM). We conducted a retrospective observational study to assess incidence of SPMs in patients with primary CM and to identify associated risk factors. METHODS: Cases of first primary CM diagnosed from 2000 to 2018 were extracted from the national cancer database Surveillance, Epidemiology, and End Results Program. Standardized incidence ratios (SIR) and excess absolute risk (EAR) of SPMs were calculated compared to a matched cohort from the general population with similar sex, race, age group, and calendar year. EAR was per 10,000 individuals, and a P-value of <0.05 was considered significant. RESULTS: A total of 471 patients met inclusion criteria, 57 (12.1%) of whom developed second primary malignancies (excluding eye and orbit melanomas) over an average (±SD) follow-up period of 6.8 (±5.0) years. Average age at diagnosis for the overall cohort was 60.2 (±18.6) years. Patients with CM demonstrated a significantly increased risk for overall SPMs relative to the general population, even after excluding eye and orbit melanomas (SIR 1.52; 95% confidence interval [CI], 1.15-1.97; EAR 67.58). Specific sites and malignancy types with increased risk were cutaneous melanoma (SIR 7.95; 95% CI, 4.45-13.12; EAR 45.34), ophthalmic non-melanoma malignancies (SIR 80.92; 95% CI, 2.05-450.84; EAR 3.41), and non-intrahepatic biliary malignancies (SIR 11.72; 95% CI, 1.42-42.32; EAR 6.32). Risk of overall SPMs (excluding eye and orbit melanomas) was significantly increased 5-10 years from diagnosis date. CONCLUSIONS: Patients with CM had an increased incidence of SPMs compared to the general population. Specifically, these patients developed more cutaneous, ophthalmic non-melanoma, and non-intrahepatic biliary malignancies. These second neoplasms could be due to shared pathophysiology or mutual risk factors. Patients with CM may benefit from surveillance for SPMs, such as annual age-appropriate screenings in the first 10 years after diagnosis.