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Background: To review the characteristics of all Slovenian patients with ocular adnexal lymphoma (OAL) in the period of 24 years with the aim of evaluating demographic data, lymphoma location and type, disease stage, treatment modality, local control rate and survival rate., Patients and Methods: All patients with histologically diagnosed OAL in the main tertiary centre of Slovenia, Eye Hospital, University Medical Centre Ljubljana, who were treated at Institute of Oncology Ljubljana were included in the study. Patients' data were collected from October 1995 through April 2019., Results: Seventy-four patients were included in the study having a median age of 68 years at diagnosis. The majority of lymphomas were of B-cell origin (98.6%). The most frequent type was the extranodal marginal zone B-cell lymphoma (MALT) (71.6%). Orbital lymphomas were diagnosed in 56 cases (75.7%) and conjunctival in 18 cases (24.3%). Ocular manifestation was the first sign of the disease in 78.4% of patients and in 67.6% of patients ocular adnexa were the only disease location. Fifty-one patients (68.9%) were treated with radiotherapy, 7 patients (9.4%) with systemic treatment, 5 patients (6.8%) with combined radiotherapy and systemic treatment and in 11 patients, biopsy and active surveillance strategy was applied (14.9%). Local control of the disease was achieved in 96.6% of treated patients. Median overall survival of the whole study group has not been reached yet. Five-year overall survival rate was 80.1% (95% CI 68.1% - 88.5%) and 5-year lymphoma specific survival rate was 87.2% (95% CI 83.2%-91.2%)., Conclusions: OALs comprise a group of heterogeneous diseases with variable outcomes depending predominately on the patient's age and lymphoma type, with low grade lymphomas carrying good prognosis even in elderly patients., (© 2024 Lucka Boltezar et al., published by Sciendo.)

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).)

Introduction: Cutaneous malignant melanomas rarely occur in the eye, usually in the eyelids or the conjunctiva. Conjunctival malignant melanomas are even rarer. Most melanomas are dark in color as they are pigmented. However, amelanotic conjunctival malignant melanomas, a scarce variant of the cancer, can be challenging to diagnose accurately., Case Presentation: We present two cases of white Caucasian Swedish-born women who were diagnosed with unilateral amelanotic malignant melanoma in the conjunctiva of the eye. In the first case, the patient was an 81-year-old woman who was suffering from redness and foreign body sensation in the left eye. The initial diagnosis was blepharitis. Three biopsies were taken, which showed malignant melanoma in the eyelid and the conjunctiva. Unfortunately, the eye and the rest of the orbit could not be saved, and the patient had to undergo an orbital exenteration. In the second case, the patient was a 50-year-old woman, and the tumor was localized in the temporal conjunctiva of the left eye. The initial diagnosis was pinguecula, but at the time of surgery, the physician suspected conjunctival intraepithelial neoplasia. The tumor was not completely removed, so adjuvant brachytherapy and local chemotherapy were used. The eye was preserved. No neck and/or lung metastasis was detected in either case at the time of diagnosis., Conclusions: Conjunctival amelanotic malignant melanomas should be suspected when tumors are present in the eye and/or the eyelids. By suspecting amelanotic malignant melanoma, the delay in treatment can be shortened. Treating them as soon as possible is essential to minimize the risk of metastasis., (© 2024. The Author(s).)

A 92-year-old woman presented with a large bulbar conjunctival mass in the OD. She also had a palpable parotid mass which on fine needle aspiration biopsy confirmed to be metastatic squamous cell carcinoma. The conjunctival mass was biopsied to confirm the diagnosis of squamous cell carcinoma with positive programmed cell death ligand 1 expression and a high tumor mutation burden. She was treated with pembrolizumab and had complete resolution of the conjunctival mass and the associated parotid metastasis after just 2 cycles of treatment. This case underscores the promising role of immune checkpoint inhibitors in the treatment of conjunctival squamous cell carcinoma, especially when surgery is associated with significant ocular morbidity, in patients who may not be good surgical candidates, or in patients with metastasis., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: The purpose of this study was to describe the response of a papillomatous ocular surface squamous neoplasia (OSSN) to the intramuscular (IM) 9-valent human papillomavirus (HPV) vaccine after failed medical and surgical interventions., Methods: A 79-year-old White man with a conjunctival lesion underwent a biopsy which revealed OSSN and positivity for high-risk HPV. Initially treated with medical therapy and surgical excisions, the patient developed a recurrence and refused further surgery. He was given 4 doses of IM HPV vaccine at the 6-week interval., Results: A dramatic reduction in lesion size and reduced epithelial thickening and hyperreflectivity was noted on slitlamp examination and high-resolution anterior segment optical coherence tomography after receiving the IM HPV vaccine. Although lesion size was markedly reduced, the therapy did not achieve total resolution, resulting in further treatment with topical 1% 5-fluorouracil (5-FU) eye drops and later 0.04% mitomycin C eye drops. The patient then elected to discontinue further treatment and solely observe., Conclusions: This case report adds to the growing literature demonstrating the potential therapeutic use of vaccines in cancer treatment. Although HPV vaccination is currently approved for prophylaxis, the use of HPV vaccines as a therapeutic option for various HPV-mediated diseases, including OSSN, should be further explored. The HPV vaccine yielded significant initial improvement in this patient who refused further surgical interventions. The use of IM HPV vaccine as an adjunctive treatment of papillomatous OSSN may represent a potential therapeutic option in cases refractory to standard treatment modalities., Competing Interests: Drs. C. L. Karp and A. Galor have a pending PCT/US2022/029842 with the University of Miami. Dr. C. L. Karp is on the medical advisory board for Glaukos. Dr T. Ioannides holds patent 10 799 574 for the use of human papillomavirus vaccines for the treatment of malignant lesions. The remaining authors have no conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Conjunctival melanoma, a rare malignancy of the ocular surface, is increasing in incidence. When small, straightforward excision with "no touch" surgery and cryotherapy at an experienced centre can provide excellent outcomes. When advanced, management is more complex and highly individualized. The risk of metastatic disease from conjunctival melanoma is as high as 30% and depends on tumour origin, American Joint Committee on Cancer (AJCC) classification, biomarkers, and perhaps most important, management technique. Metastatic disease can result in melanoma-associated death. Therefore, early detection and prompt directed treatment at an experienced centre are important for protection from metastasis. In this review, we provide an update on conjunctival melanoma clinical features, diagnostic modalities, AJCC staging, genetic markers, and the most critical, controlled management with minimization of tumour seeding. We detail the new era of characterization of conjunctival melanoma with molecular biomarkers that predict melanoma prognosis. This could lead to precision medicine with targeted approaches to specific mutations that improve patient survival. As we work together, the field of conjunctival melanoma is moving forward., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Uveal and conjunctival melanomas are relatively rare tumors; nonetheless, they pose a significant risk of mortality for a large number of affected individuals. The pathogenesis of melanoma at different sites is very similar, however, the prognosis for patients with ocular melanoma remains unfavourable, primarily due to its distinctive genetic profile and tumor microenvironment. Regardless of considerable advances in understanding the genetic characteristics and biological behaviour, the treatment of uveal and conjunctival melanoma remains a formidable challenge. To enhance the prospect of success, collaborative efforts involving medical professionals and researchers in the fields of ocular biology and oncology are essential. Current data show a lack of well-designed randomized clinical trials and limited benefits in current forms of treatment for these tumors. Despite advancements in the development of effective melanoma therapeutic strategies, all current treatments for uveal melanoma (UM) and conjunctival melanoma (CoM) remain unsatisfactory, resulting in a poor long-term prognosis. Ongoing trials offer hope for positive outcomes in advanced and metastatic tumors. A more comprehensive understanding of the genetic and molecular abnormalities involved in the development and progression of ocular melanomas opens the way for the development of personalized therapy, with various potential therapeutic targets currently under consideration. Increased comprehension of the molecular pathogenesis of UM and CoM and their specificities may aid in the development of new and more effective systemic therapeutic agents, with the hope of improving the prognosis for patients with metastatic disease., Competing Interests: The authors declare that they have no conflicts of interest to report regarding the present study., (© 2024 Kaštelan et al.)

Lymphomas of the eye and ocular adnexa are rare lymphoproliferative diseases of the ocular and ocular adnexal tissue. The incidence of these diseases has been rapidly increasing over the past few decades. The exact pathogenesis remains unknown, but it is postulated to be multifactorial and includes genetic aberrations, epigenetic and environmental factors, infectious agents, and chronic antigenic stimulation. The majority of ocular and ocular adnexal lymphomas are of B-cell origin, except for eyelid lymphomas, which are more often of T-cell type. Lymphoproliferative diseases of ocular and ocular adnexal structures are either primary, when they arise in the eye, orbit, lacrimal gland, eyelid, and/or conjunctiva, or secondary extranodal manifestation of systemic lymphoma. Diagnosis is challenging and requires a multidisciplinary approach involving ophthalmologists, dermatologists, oncologists, and radiation oncologists., Competing Interests: Declaration of competing interest The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

To elucidate long-term outcome in primary conjunctival lymphoma, a review was conducted of 31 consecutive patients: 21 men and 10 women with an age range of 28 to 85 (median, 61) years at presentation and follow-up periods ranging from 1 to 19 (median, 7) years. Conjunctival lymphoma was on the right side in 10 patients, on the left side in 12, and on both sides in 9. Upper, lower, or both fornix lesions in 28 patients were all diagnosed as extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), while thick nasal bulbar conjunctival lesions in 3 patients were differently diagnosed as MALT lymphoma, diffuse large B-cell lymphoma, and follicular lymphoma, respectively. Seven patients underwent local radiation (30 Gy): as initial treatment in 5 patients and treatment for relapse in 2 patients. The remaining 24 patients were observed without additional treatment after excisional biopsy: 5 of these 24 patients showed relapse 0.5 to 6 years later and underwent excisional biopsy again that revealed MALT lymphoma. Of the 5 patients with relapse, only one with second-time relapse underwent radiation. Fluorodeoxyglucose positron emission tomography was performed in 18 patients and showed no systemic lesions: high uptake was noted in the residual conjunctival lesions of 4 patients and in the relapsed conjunctival lesions of 3 patients. One patient died of rectal cancer while no patients died of lymphoma. Observation is an option in patients with primary conjunctival lymphoma after excisional biopsy. Radiation is a treatment option in the case of relapse.

Background: In sub-Saharan Africa, ocular surface squamous neoplasia (OSSN) is the most common ocular surface tumour and is strongly associated with HIV infection. This range of ocular malignancies can be managed early to prevent large tumours requiring invasive treatment, facial disfigurement and mortality. Primary healthcare workers (HCWs) play a critical role in the early identification of the lesion. In addition, the ocular lesion can also be the presenting sign of HIV infection in individuals who have not yet been diagnosed. The aim of the present study was to assess the management of suspicious conjunctival growths in HIV-infected patients in primary health facilities and a specialist eye clinic in South Africa., Objectives: To assess the knowledge, attitude and current practice of HCWs working in HIV clinics regarding ocular surface lesions and to evaluate the management of patients with ocular surface lesions at a tertiary hospital., Methods: A cross-sectional study design was used (November 2020 - May 2021), for which 149 HCWs were invited to assessments about their knowledge, attitudes and practices regarding ocular surface lesions. In addition, files of patients with ocular surface lesions who presented between January 2018 and August 2020 to the eye clinic were reviewed using a data extraction sheet., Results: One hundred-and-three HCWs agreed to participate in the survey (response rate 69.1%). Of these participants, 84.5% were experienced professional nurses (6 - 15 years of work experience) but had minimal experience with detection and management of eye complaints and lesions. Twenty-seven (26.2%) of the participants recognised some ocular surface lesions and 86 (83.5%) reported that they would refer patients with suspicious lesions. Sixty-two files were reviewed and 51 (82.2%) of the patients had an HIV-positive diagnosis. Fifty percent had carcinoma-in situ and squamous cell carcinoma of the conjunctiva. Thirty-one (50%) of the patients were lost to follow-up., Conclusion: OSSN is an important manifestation of HIV infection. It would be beneficial for patients to receive a basic ocular examination as part of the baseline clinical evaluation; this may contribute to early referral to an eye care facility. The health system would benefit from establishing an eye health support system with the nearby health facilities, thereby educating primary HCWs about the association between HIV and OSSN.

Background/objectives: Ocular surface squamous neoplasia (OSSN) are among the most frequent non-pigmented malignancies of the ocular surface. They have a wide range of histological characteristics - ranging from mild epithelial dysplasia to invasive carcinoma of the squamous cells of the cornea. They may be restricted to the conjunctiva or also involve the cornea. As there are no leading symptoms in the early stages, diagnosis may be very delayed in patients who do not receive regular ophthalmological treatment. The present case series describes clinical and histological data on OSSN and includes clinical and histological data on OSSN, including possible clinical presentations, important risk factors, special histological and cytological features and therapeutic options., Methods: Retrospective case series of patients with histologically confirmed severe epithelial dysplasia of the conjunctiva and cornea consistent with OSSN who presented to the Department of Ophthalmology in Basel University Hospital. The analysis covered demographic data, symptoms, diagnostic testing (photo documentation, brush biopsy), treatment and cytological and/or histological material and findings., Results: We report on five patients aged between 41 and 92 years at the time of diagnosis. The histological findings in all patients included severe epithelial dysplasia, but with a heterogenous clinical presentation. In all cases, the lesion started in the conjunctiva, but traversed the limbus and extended to the cornea. The primary treatment was always surgical removal. In one patient, this had to be repeated several times due to recurrent metaplasia and was complemented by subsequent mitomycin C therapy. The clinical outcome ranged between total restitution of the original state to inevitable enucleation., Conclusion: The clinical presentation of OSSN is highly heterogenous, so that the initial diagnosis is difficult. There are no official guidelines for treatment, so that the treatment of choice varied between clinics. Regular ophthalmological follow-ups are recommended, even after complete surgical excision. Possible relevant concomitant diseases and risk factors must be identified before therapy., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Purpose: To review the outcomes of targeted therapy and immunotherapy in advanced conjunctival tumors, including conjunctival squamous cell carcinoma, conjunctival melanoma, and conjunctival lymphoma., Methods: A Pubmed database systematic search was performed between January 1999 and December 2022. The literature search was limited to studies published in English., Results: This review included 142 patients with advanced malignant conjunctival tumors from 42 articles. In the conjunctival squamous cell carcinoma group, 2 cases of advanced conjunctival squamous cell carcinoma treated with epidermal growth factor receptor inhibitors showed significant tumor size improvement after 7.5 months of follow-up. Among 7 cases treated with systemic immunotherapy, 5 cases (72%) had complete response (CR), 1 case (14%) showed partial response (PR), and 1 case (14%) had stable disease (SD) after 16 months. In the conjunctival melanoma group, among 18 cases treated with combined v-raf murine sarcoma viral oncogene homolog B1/mitogen-activated extracellular signal-regulated kinase inhibitors, 6 (33%) had CR, 5 (28%) had PR, 2 (11%) had SD, and 5 (28%) had progressive disease after 24.8 months of follow-up. Of 44 conjunctival melanoma cases treated with immunotherapy, 12 (28%) had CR, 9 (20%) had PR, 7(16%) had SD, and 16 (36%) had progressive disease after 14.2 months. Systemic Rituximab treatment for conjunctival lymphoma cases resulted in CR in 21 patients (63%), PR in 11 patients (33%), and SD in 1 patient (3%) after 20.5 months of follow-up. Intralesional Rituximab injections in 38 conjunctival lymphoma cases showed CR in 28 patients (75%), PR in 7 patients (19%), SD in 1 patient (2%), and progressive disease in 2 patients (4%) after 20.4 months of follow-up., Conclusions: Despite limited clinical case reports and short-term follow-ups, targeted therapy and immunotherapy have shown promising results for advanced malignant conjunctival tumors., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To evaluate clinical and demographic characteristics and factors affecting recurrence, metastasis, and survival in conjunctival melanoma (CM)., Methods: The clinical records of 45 patients who were treated for CM between October 1998 and June 2022 were retrospectively evaluated. Age, gender, presence of underlying conjunctival nevus-primary acquired melanosis (PAM), tumor stage according to the 8th edition of the American Joint Committee on Cancer (AJCC) staging system, tumor basal diameter, tumor thickness, lymph node (LN) involvement, metastasis, presence of tumor at the surgical margin, treatment method, need for adjuvant therapy, local tumor control, recurrence, and survival were recorded., Results: Twenty-one (46.7%) patients were female and 24 (53.3%) patients were male. The mean age at diagnosis was 53.2 ± 16.1 years. Median follow up time was 12 (1-300) months. Fifteen (33.3%) patients had conjunctival PAM; 2 (4.4%) patients had conjunctival nevus. The tumor stage was T1 in 24 (55.8%), T2 in 13 (30.2%), and T3 in 6 (14.0%) of the cases. The T stage in 2 cases could not be determined. For stage T1 and T2 CM, in addition to excisional biopsy (EB) and cryotherapy, alcohol epitheliectomy (AE) was performed in 17 cases (37.8%), superficial sclerectomy (SS) was performed in 7 (15.6%), and amnion membrane transplantation (AMT) due to a large conjunctival defect in 9 (20.0%). Six (14.0%) T3 cases underwent primary exenteration. Positive surgical margins were observed in 23 (51.1%) of the excised tumors at histopathologic examination. Adjuvant topical mitomycin-C (MMC) was used in 7 (30.4%) and strontium-90 episcleral brachytherapy in 4 (17.4%) of the 23 cases with tumor-positive borders. During the follow-up, recurrence was seen in 14 (31.1%) cases. According to Kaplan Meier analysis, the mean time to recurrence development was 90.5 ± 16.1 months and the 5-year recurrence free rate was 52.0%. Fourteen of the recurrent cases underwent EB + cryotherapy, 3 underwent AE + SS, and 3 underwent secondary exenteration. Metastasis and LN involvement occurred in 11 (24.4%) and 8 (17.8%) of the cases, respectively. Four (8.9%) cases expired during follow-up. According to Kaplan-Meier analysis, the mean time to metastasis was 106.2 ± 17.3 months and the 5-year metastasis free rate was 52.0%. While recurrence was more frequent in CM developing from PAM/nevus, metastasis was more frequent in men and those with LN involvement., Conclusion: Conjunctival melanoma was a malignant tumor with high recurrence and metastasis rates. Precursor nevus/PAM is a risk factor for recurrence, while male gender and regional LN involvement were risk factors for metastasis in this study., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Lymphoma of the conjunctiva is an ocular malignancy derived from clonal proliferation of lymphocytes. The majority of conjunctival lymphoma is extranodal marginal zone B-Cell lymphoma (EMZL), however diffuse large B-cell (DLBCL), follicular (FL), mantle cell (MCL) and T- cell subtypes are also seen. Clinical manifestations are non-specific, but include unilateral or bilateral painless salmon-pink conjunctival lesions. Approaches to treatment have centered around local immunomodulation, often with Interferon-α2b or Rituximab (anti-CD20 monoclonal antibody) with or without radiation. Although conjunctival lymphoma is generally considered an indolent disease, recent advances in next-generation sequencing have improved clinicians' ability to predict future recurrence or systemic disease through assessment of cytogenic and molecular features. In this paper, we review the classification, clinical features, diagnostic techniques, and emerging strategies for management and prognostication of conjunctival lymphomas., (© 2022. The Author(s).)

Purpose: This study was taken up to look into the various causes of recurrence, clinicopathological profile and final outcomes in recurrent cases of ocular surface squamous neoplasia (OSSN)., Methodology: A prospective cohort study was conducted and total 18 patients were recruited. All patients were subjected to detailed history, comprehensive eye examination and imaging studies. A treatment plan was formulated based on the size and extent of the lesion. The primary outcome measure was complete response to treatment with no evidence of recurrence after12 months and secondary outcome measure was complication associated with each treatment modality., Results: The common cause of recurrence in our study was misdiagnosis of the lesion as in 15 cases it was diagnosed as pterygium and in 03 cases it was actinic keratosis. Excision Biopsy with adjunctive cryotherapy was the preferred treatment modality followed by topical interferon-alpha 2b drop-in our study. We could achieve good outcomes in terms of complete response to the treatment in 16 cases. The complication associated with this treatment was minimal in our study as few patients complained redness and irritation which subsided after application of topical lubricants. There was no sign of recurrence even at 12 months of follow-up in all 18 cases., Conclusion: The current study provided clinicopathological characteristics and treatment outcomes in recurrent cases of OSSN. In our study, adopting appropriate treatment strategy, regular follow-up to assess the response to treatment and change over to new treatment plan in cases with inadequate response helped in achieving good outcomes in recurrent cases of OSSN., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Conjunctival tumors result from gain of tissue, which can be either degenerative or neoplastic, but also inflammatory. In this article, degenerative (pterygium and pinguecula) as well as benign and malignant neoplastic conjunctival changes (epithelial, melanocytic and vascular tumors, choristomas as well as metastases) are discussed with regard to pathogenesis, symptoms, diagnostics and current status of treatment., Competing Interests: Erklärung zu finanziellen Interessen Forschungsförderung erhalten: nein; Honorar/geldwerten Vorteil für Referententätigkeit erhalten: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an im Bereich der Medizin aktiven Firma: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an zu Sponsoren dieser Fortbildung bzw. durch die Fortbildung in ihren Geschäftsinteressen berührten Firma: nein. Erklärung zu nichtfinanziellen Interessen Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

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.)

Conjunctival lymphomas are rare entities and may present with non-specific ocular signs that resemble inflammation. They may mimic common ocular pathologies, leading to a delay in diagnosis and treatment. The treatment options of conjunctival lymphomas should be tailored to individuals due to their indolent nature compared to other adnexal lymphomas. Herein, the authors report a case of a primary follicular conjunctival lymphoma in a patient who presented with signs and symptoms of nodular anterior scleritis. The final histology of the conjunctiva lesion revealed primary follicular lymphoma. The patient was managed conservatively with active surveillance., (© 2022 Marshfield Clinic Health System.)

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).)

Background: Conjunctival myeloid sarcoma (MS) as an isolated presentation of acute myeloid leukemia (AML) relapse is rare. Here, we report a case of unilateral conjunctival MS revealed as a sign of AML relapse., Case Presentation: A 50-year-old man with a history of AML in remission visited our clinic presenting with a left conjunctival injection persisting for 1 month. Diffuse subconjunctival thickening with conjunctival vascular engorgement was observed. Ultrasound biomicroscopy revealed a hyper-reflective, thickened conjunctiva in his left eye. During the incisional biopsy, the lesion was strongly attached to the underlying sclera; histopathologic examination revealed infiltration of leukemic blasts. The relapse of AML was confirmed by a successive bone marrow biopsy. The ocular lesion disappeared after allogeneic peripheral blood stem cell transplantation (PBSCT) and concomitant salvage radiotherapy on the left eye. The patient has remained in remission for 3 years after allogeneic PBSCT., Conclusions: Incidental conjunctival lesions can indicate AML relapse in patients treated earlier for AML. An ophthalmologist may have a role in the early detection of AML when a patient presents with an atypical conjunctival lesion., (© 2022. The Author(s).)

Conjunctival melanoma (CM) accounts for 5% of all ocular melanomas and arises from malignantly transformed melanocytes in the conjunctival epithelium. Current therapies using surgical excision in combination with chemo- or cryotherapy still have high rates for recurrences and metastatic disease. Lately, novel signal transduction-targeted and immune checkpoint inhibitors like cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) inhibitors, programmed cell death protein-1 (PD-1) receptor inhibitors, BRAF- or MEK-inhibitors for systemic treatment of melanoma have improved the outcome even for unresectable cutaneous melanoma, improving patient survival dramatically. The use of these therapies is now also recommended for CM; however, the immunological background of CM is barely known, underlining the need for research to better understand the immunological basics when treating CM patients with immunomodulatory therapies. Immune checkpoint inhibitors activate tumor defense by interrupting inhibitory interactions between tumor cells and T lymphocytes at the so-called checkpoints. The tumor cells exploit these inhibitory targets on T-cells that are usually used by dendritic cells (DCs). DCs are antigen-presenting cells at the forefront of immune response induction. They contribute to immune tolerance and immune defense but in the case of tumor development, immune tolerance is often prevalent. Enhancing the immune response via DCs, interfering with the lymphatic pathways during immune cell migration and tumor development and specifically targeting tumor cells is a major therapeutic opportunity for many tumor entities including CM. This review summarizes the current knowledge on the function of lymphatic vessels in tumor growth and immune cell transport and continues to compare DC subsets in CM with related melanomas, such as cutaneous melanoma and mucosal melanoma.

Purpose: To describe the risk factors, clinical presentation, management, and outcomes of patients with bilateral ocular surface squamous neoplasia (OSSN)., Methods: Retrospective case series., Results: Of the 25 patients with bilateral OSSN, the mean age at diagnosis of OSSN was 31 years (median, 24 years; range, 2-60 years). Risk factors for bilateral OSSN included xeroderma pigmentosum ( n  = 15, 60%), human immunodeficiency virus infection ( n  = 3, 12%), conjunctival xerosis ( n  = 1, 4%), and topical steroid use ( n  = 1, 4%). There were no identifiable ocular or systemic risk factors in 7 (28%) patients. Presentation was synchronous in 14 (56%) and metachronous in 11 (44%) patients. Tumor morphology was bilaterally similar in 12 (48%) patients. Histopathological examination ( n  = 36) revealed conjunctival intraepithelial neoplasia (CIN) grade 1 in 4 (8%); grade 2 in 7 (14%); carcinoma in situ in 5 (10%), and invasive carcinoma in 20 (40%). Primary management of OSSN ( n  = 49) included excisional biopsy ( n  = 31, 62%), topical immunotherapy (IFN α2B) ( n  = 11; 22%), topical Mitomycin C (MMC) ( n  = 3, 6%), enucleation ( n  = 1, 2%), orbital exenteration ( n  = 2, 4%), and plaque brachytherapy (PBT) ( n  = 1, 2%). One patient was lost to follow-up after detection of tumor in the second eye. Recurrent tumors were noted in 16 (32%) eyes and binocular globe salvage was achieved in 16 (64%) patients at a mean follow up of 41 months (median 30 months; range, 1-164 months)., Conclusion: OSSN occurrence can be synchronous or metachronous. Meticulous examination of the fellow eye is important for an early diagnosis of OSSN.

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.)

Introduction: Ocular adnexal lymphoma and vitreoretinal lymphoma are rare forms of non-Hodgkin lymphoma. They are regarded as distinct disease entities due to the differences in molecular mechanism, management, and outcome. We present a rare case of conjunctival diffuse large B cell lymphoma (DLBCL) that developed to vitreoretinal lymphoma after systemic chemotherapy., Patient Concerns: A 60-year-old man presented with a left salmon-colored conjunctival mass., Diagnosis: A biopsy was performed, and histopathologic examination showed DLBCL. Immunohistochemical staining was positive for CD20 with increased κ to λ light chain ratio., Interventions: Bone marrow biopsy also revealed DLBCL. Gallium-67 scintigraphy showed abnormal uptake only in the left orbital lesion. Ann Arbor stage was estimated as IV. The patient underwent systemic combination chemotherapy and immunotherapy., Outcomes: Four months after the last course of chemotherapy, primary conjunctival DLBCL relapsed, manifesting vitreous opacity. Diagnostic vitrectomy confirmed a diagnosis of vitreoretinal lymphoma., Lessons: Conjunctival DLBCL and vitreoretinal lymphoma are both DLBCL. After systemic chemotherapy for conjunctival DLBCL, the lymphoma may relapse in intraocular sites as secondary vitreoretinal lymphoma., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Lacrimal drainage apparatus melanoma is a rare entity that may arise primarily or, more commonly, as secondary involvement from melanoma originating elsewhere. Conjunctival melanoma may involve the lacrimal drainage apparatus (LDA) via spread along the canalicular epithelium, separate in situ processes, or direct invasion. Only seven cases exist in the literature where conjunctival melanoma remotely recurred in the LDA. We report three additional patients, two with invasive conjunctival melanoma and one with primary acquired melanosis (PAM) with severe atypia/melanoma in situ, who developed LDA melanoma at 5, 8, and 16 years after initial treatment of conjunctival melanoma. This report confirms the ability of conjunctival melanoma to give rise to spatially and temporally remote LDA melanoma despite adequate local treatment, and reviews the proposed mechanisms and associated characteristics of LDA recurrence in conjunctival melanoma.

Background: To relate conjunctival melanoma characteristics to local control., Methods: Retrospective, registry-based interventional study with data gathered from 10 ophthalmic oncology centres from 9 countries on 4 continents. Conjunctival melanoma patients diagnosed between January 2001 and December 2013 were enrolled in the study. Primary treatments included local excision, excision with cryotherapy and exenteration. Adjuvant treatments included topical chemotherapy, brachytherapy, proton and external beam radiotherapy (EBRT). Cumulative 5-year and 10-year Kaplan-Meier local recurrence rates were related to clinical and pathological T-categories of the eighth edition of the American Joint Committee on Cancer (AJCC) staging system., Results: 288 patients had a mean initial age of 59.7±16.8 years. Clinical T-categories (cT) were cT1 (n=218,75.7%), cT2 (n=34, 11.8%), cT3 (n=15, 5.2%), cTx (n=21,7.3%) with no cT4. Primary treatment included local excision (n=161/288, 55.9%) followed by excision biopsy with cryotherapy (n=108/288, 37.5%) and exenteration (n=5/288, 1.7%). Adjuvant therapies included topical mitomycin (n=107/288, 37.1%), plaque-brachytherapy (n=55/288, 19.1%), proton-beam (n=36/288, 13.5%), topical interferon (n=20/288, 6.9%) and EBRT (n=15/288, 5.2%). Secondary exenteration was performed (n=11/283, 3.9%). Local recurrence was noted in 19.1% (median=3.6 years). Cumulative local recurrence was 5.4% (3.2-8.9%), 19.3% (14.4-25.5%) and 36.9% (26.5-49.9%) at 1, 5 and 10 years, respectively. cT3 and cT2 tumors were twice as likely to recur than cT1 tumours, but only cT3 had statistically significantly greater risk of local recurrence than T1 (p=0.013). Factors such as tumour ulceration, plica or caruncle involvement and tumour thickness were not significantly associated with an increased risk of local recurrence., Conclusion: This multicentre international study showed that eighth edition of AJCC tumour staging was related to the risk of local recurrence of conjunctival melanoma after treatment. The 10-year cumulative local recurrence remains high despite current management., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht.

Ocular surface squamous neoplasia (OSSN) is the most common ocular tumour with an incidence ranging from 0.03 to 1.9 per 100,000 persons/year. The diagnosis is made on clinical suspicion and confirmed with anterior-segment optical coherence tomography (AS-OCT), cytology, or histology. The purpose of this review is to provide an overview of the management options available for OSSN and review their success and recurrence rates. Surgery is the gold standard for the management of small OSSN lesions. With the increased use of less invasive diagnostic modalities such as AS-OCT and cytology, there has been a move to use topical therapies for the management of OSSN. The most commonly used agents are interferon-α2b (IFN), mitomycin-C (MMC) and 5-fluorouracil (5FU). They have been shown to have similar resolution and recurrence rates but differ in cost and side effect profile. IFN has the lowest side effect profile, but is also the most expensive, whereas MMC has the greatest surface toxicity and is priced midway between the three. 5FU is the cheapest of the three topical agents with less surface toxicity than MMC. Radiotherapy is mostly employed as adjuvant therapy. Newer novel therapies are available but have not been widely adopted as mainstream therapy due to cost and lack of clinical evidence. OSSN has the benefit of many management options. No single modality has been shown to superior and some patients will need the use of combination therapy to achieve an optimal clinical outcome.

Conjunctival melanoma (CM) is an ocular malignant tumor arising from the bulbar and palpebral conjunctiva and from the caruncle. The treatment of early-stage CM is wide local excision, followed by cryotherapy to the margins and adjuvant therapy postoperatively. Advanced CM has a poor prognosis, and there is no consensus on its management. With the development of precision medicine, the identification of genetic alterations assumes great importance. The genetic characteristics of CM, such as BRAF, NRAS, and NF1 mutations, may provide potential therapeutic targets. For locally advanced tumors and metastatic disease, targeted therapy such as BRAF inhibitors and MEK inhibitors in vitro show therapeutic benefit. Some individual case reports indicate their potential effectiveness in advanced CM. In addition, immune checkpoint inhibitors, such as programmed cell death-1 and cytotoxic T lymphocyte antigen-4 inhibitors, have been successfully used for advanced cutaneous melanoma and may be effective in CM. Limited clinical case reports found immune checkpoint inhibitors effective in advanced CM. More clinical studies are needed., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Conjunctival melanoma is a rare disease which requires tailored management in most cases. The mainstays of treatment can be classified as surgery, topical chemotherapy, radiotherapy, cryotherapy, and other emerging treatment modalities. Herein we review conventional approaches as well as more recently introduced treatment options, together with advances in molecular biology in this particular disease.

Purpose: The aim of this study was to evaluate outcomes of conjunctiva melanoma based on the American Joint Committee on Cancer Classification (AJCC) 8th edition., Design: Retrospective interventional case series., Methods: Outcomes analysis of 425 patients., Results: In this analysis of 425 patients with conjunctival melanoma, there were 266 (63%) patients classified as T1, 75 (18%) as T2, 84 (20%) as T3, and 0 (0%) as T4. A comparison (T1 vs T2 vs T3) revealed that history of primary acquired melanosis was more common in T2 (81% vs 96% vs 81%; P = 0.01) and conjunctival nevus more common in T1 (20% vs 9% vs 11%; P = 0.03). Of 381 patients with follow-up (mean of 57.6 months), comparison revealed higher T category with increasing local recurrence/new tumor (30% vs 43% vs 49%; P = 0.004), increasing exenteration (3% vs 9% vs 28%; P < 0.001), increasing melanoma-related locoregional lymph node metastasis (2% vs 7% vs 12%; P = 0.001), increasing melanoma-related systemic metastasis (9% vs 25% vs 23%; P < 0.001), and increasing melanoma-related death (4% vs 12% vs 18%; P < 0.001). A comparison at 10 years revealed visual acuity loss of >3 lines (32% vs 42% vs 63%; P < 0.001), melanoma recurrence/new tumor (47% vs 70% vs 74%; P < 0.001), exenteration (4% vs 24% vs 46%; P < 0.001), melanoma-related locoregional lymph node metastasis (3% vs 13% vs 25%; P < 0.001), melanoma-related systemic metastasis (13% vs 45% vs 40%; P < 0.001), and melanoma-related death (8% vs 22% vs 37%; P < 0.001)., Conclusions: Based on the AJCC 8th edition of conjunctival melanoma, the 10-year risk per T category significantly increased for visual acuity loss of >3 lines, recurrence/new tumor, exenteration, locoregional and systemic melanoma-related metastasis, and melanoma-related death., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

Purpose : To describe ocular surface disorders associated with human immunodeficiency virus (HIV). Methods : Narrative review. Results : Ocular surface neoplastic conditions, such as Kaposi's sarcoma, conjunctival lymphoma and ocular squamous cell carcinoma along with blepharitis, dry eye disease, and vernal keratoconjunctivitis, constitute ocular surface complications in HIV-infected individuals. Conclusion : This review will provide a summary of clinical presentations and treatment options for the most common HIV-related ocular surface diseases, indicating the need for a comprehensive ocular examination including ocular surface in all HIV patients 22.

Multiple myeloma (MM) is the second most prevalent hematologic malignancy after non-Hodgkin lymphoma and is currently considered incurable. Clinical ophthalmic manifestations of MM are rare but at the same time diverse. Ocular surface manifestations of multiple myeloma are uncommon. Conjunctival 'salmon patch' is a typical ocular surface ophthalmological sign with a distinct set of differential diagnoses, including most often ocular adnexal lymphoma. This case report presents a 33-year-old female with a relapse of MM manifesting as a conjunctival 'salmon patch'. The patient initially responded well to medical management including high dose melphalan supported by a third autologous stem cell transplantation (ASCT) and did not require further surgical excision of the ocular lesion. It is suggested that MM should be included in the differential diagnosis of 'salmon patch' conjunctival lesions.

The aim of this paper is to give an overview of different benign and malignant epibulbar tumors. Categories can be made of the age of manifestation (paediatric tumors versus tumors of adults), the originating cell types (squamous cell tumors, melanocytic tumors, lymphomas or germ cell tumors) or genesis (proliferative, infectious or degenerating tumors). Most epibulbar tumors show lesions on the conjunctiva or the cornea. These can be flesh coloured or pigmented efflorescences with leucoplakias or hyperkeratosis. Especially malignant tumors show atypical growth of blood vessels, are often prominent and can have ulcerations or bleeding. In case of tumor growth, change in pigmentations or atypical bleedings a malign tumor can be suspected. The diagnosis should be confirmed using histopathological evaluation after an incisional or excisional biopsy. Molecular pathologic techniques extend the diagnostic tools and have an implication on the therapy of metastatic tumors. Therapeutic options of the malignant tumors (apart from lymphomas) are chirurgical excision preferably with a no touch technique and afterwards an adjuvant therapy consisting of radiation or cryotherapy. A topical chemotherapy with Mitomycin C, 5-Fluorouracil or Interferon α2b can be effective to reduce persisting precancerosis and reduce recurrences. In case of a suspected malignant tumor it is advisable to refer the patient to an ophthalmo-oncologic center to plan and ensure interdisciplinary therapy., Competing Interests: Erklärung zu finanziellen Interessen Forschungsförderung erhalten: nein; Honorar/geldwerten Vorteil für Referententätigkeit erhalten: nein; Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an im Bereich der Medizin aktiven Firma: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an zu Sponsoren dieser Fortbildung bzw. durch die Fortbildung in ihren Geschäftsinteressen berührten Firma: nein. Erklärung zu nichtfinanziellen Interessen Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Objectives: To find out the demography, clinical behaviour and outcome of ocular surface squamous neoplasia (OSSN) in North-west Pakistan., Study Design: Case series., Place and Duration of Study: Department of Ophthalmology, Khyber Teaching Hospital, Peshawar, Pakistan, from March 2011 to December 2017., Methodology: All patients with OSSN consulting this department were registered. A special proforma was designed to document demography, clinical features, management, and follow-up of all cases. At the end of study, the data was analysed using SPSS version 25 and the result compiled., Results: Sixty-five eyes with OSSN were included in the study, in which 40 (61.5%) were males and 25 (38.5%) were females. Mean age of the patients was 61.68 +12.76 years. Twenty-six (40%) patients were farmers and exposed to ultraviolet light for prolong period. Fifty-nine (90.8%) presented with a painless, reddish mass on ocular surface. Average size of neoplasm at presentation was 18.60 + 11.30 mm in large dimension. Histopathologically, 23 (35.4%) were carcinoma in situ and 41(63%) invasive squamous cell carcinoma. During the period of follow-up 41 (63.1%) cured, 21 (32.2%) developed recurrence, and 3 (4.6%) patients passed away., Conclusion: OSSN is common in North-west Pakistan. The presentation is late due to certain socio-economic factors; but overall behaviour of the neoplasm is almost the same as mentioned in other studies from different parts of the world. Key Words: Ocular surface squamous neoplasia (OSSN), conjunctival squamous cell carcinoma, Carcinoma in situ.

Purpose: To determine the association of Fitzpatrick skin type (FST) with conjunctival melanoma., Methods: Retrospective case series of 540 patients with conjunctival melanoma to assess clinical features and outcomes per FST., Results: The FST was Type I (n = 126, 23%), II (n = 337, 62%), III (n = 56, 10%), IV (n = 8, 2%), V (n = 12, 2%), and VI (n = 1, <1%). A comparison (FST I vs. II vs. III, IV, V, and VI) revealed Types I and II associated with older mean patient age (63.9 vs. 60.7 vs. 51.1 years, p < 0.001), greater percentage of female patients (68% vs. 44% vs. 42%, p < 0.001), lower frequency of complexion associated melanosis (1% vs. 2% vs. 13%, p < 0.001), smaller tumor thickness (2.1 vs. 2.8 vs. 3.6 mm, p = 0.01), and less eyelid involvement (13% vs. 13% vs. 28%, p = 0.02). Kaplan-Meier estimates for 5-year risk showed no difference by Types for visual acuity loss ≥3 lines, local tumor recurrence, exenteration, metastasis, or death., Conclusion and Relevance: Most patients with conjunctival melanoma show FST I or II, and this demonstrated no association with 5-year rate of vision loss, tumor recurrence, exenteration, metastasis, or death.

Conjunctival melanoma is a rare tumor of the conjunctival epithelium with a heterogenous clinical presentation and a propensity for regional and distant metastatic spread. Guidelines for the treatment of local conjunctival melanoma are well-established, but there are no standard efficacious therapies for metastatic disease. Given that conjunctival melanoma is genetically similar to cutaneous melanoma and mucosal melanomas, targeted therapies effective in the treatment of these diseases, such as BRAF inhibitors and KIT inhibitors, may be effective in the treatment of patients with metastatic conjunctival melanoma. Other targeted small-molecule drugs in the drug development pipeline for the treatment of more prevalent melanomas could also be applicable to conjunctival melanoma. Furthermore, systemic immunotherapy treatments that are now a mainstay in the treatment of cutaneous melanoma, such as programmed cell death-1 and cytotoxic T lymphocyte-associated antigen-4 inhibitors, could also stand to benefit patients with metastatic conjunctival melanoma. Limited case reports provide clues about the effectiveness of both targeted small-molecule inhibitors and immunotherapy in patients with advanced local and metastatic conjunctival melanoma and give credence to the argument that conjunctival melanoma patients should be included in major trials studying new therapies in both cutaneous and mucosal melanomas where applicable.

Purpose: To study the demographic features, treatment, histopathology, and outcomes in patients of xeroderma pigmentosum (XP) with conjunctival melanoma., Methods: Retrospective case series., Results: The median age at presentation was 18 years (range 9-30 years). There were three females and one male patient presenting with a median duration of symptoms of 3 months (range 1-60 months). The tumor was located in the bulbar conjunctiva in all 4 patients. All patients had corneal involvement by the tumor. The median tumor basal diameter was 7 mm (range 4-15 mm). Wide tumor excisional biopsy with alcohol keratoepithelectomy, cryotherapy to the free margins, and amniotic membrane grafting was done in three patients. One patient underwent orbital exenteration for extensive tumor. One patient also received adjuvant plaque brachytherapy for microscopic residual tumor. Over a median follow-up of 22 months (range 2-101 months), there were no recurrences, metastasis, or death., Conclusion: Conjunctival melanoma in XP is rare and manifests at a younger age.

Purpose: To report a rare case of adenocarcinoma (ADC) arising in the conjunctiva which locally recurred and metastasized to the periparotid lymph nodes., Methods: This is a single observational case report., Results: A 79-year-old male patient was referred to us for a suspected recurrence of conjunctival carcinoma of the right eye. At presentation, we observed an elevated conjunctival lesion with corneal involvement. He was treated with neoadjuvant mitomycin C 0.04% eye drops, followed by surgical excision of the lesion, cryotherapy of the excision margins, and reconstruction with amniotic membrane graft. The tumor was histologically diagnosed as ADC. The negative systemic evaluation and the immunoprofile led us to believe the primitive nature of the tumor. The excision margins were positive, and the patient was lost to follow up for 7 months, and when he came back, a new local recurrence was diagnosed. Then, he received rescue treatment with mitomycin C 0.04% eye drops with complete regression of the lesion. No local recurrence was observed until the 14-month follow-up visit, during which the patient complained of swelling in the right parotid region. Subsequently, he underwent total parotidectomy with neck dissection. Metastasis was found in 3 periparotid lymph nodes. The patient did not receive further treatments, and no recurrences were observed over the following 20 months., Conclusions: ADC arising in the conjunctiva is a very rare occurrence. Additional observation is required for the management of this rare conjunctival tumor.