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Purpose: To evaluate ChatGPT's ability to analyze comprehensive case descriptions of patients with uveal melanoma and provide recommendations for the most appropriate management., Design: Retrospective analysis of ocular oncology patients' medical records., Subjects: Forty patients treated for uveal melanoma between May 2019 and October 2023., Methods: We uploaded each case description into the ChatGPT interface (version 4.0) and asked the model to provide realistic treatment options by asking the question, "What type of treatment do you recommend?" The accuracy of decisions produced by ChatGPT was compared to those recorded in patients' files and the treatment recommendations provided by three ocular oncologists, each with more than 10 years of experience., Main Outcome Measures: The primary objective of this research was to assess the accuracy of ChatGPT replies in ocular oncology cases, analyzing its competence in both straightforward and intricate situations. Our secondary purpose was to assess the concordance between the responses of ChatGPT and those of ocular oncology specialists when faced with analogous clinical scenarios., Results: ChatGPT's surgical choices matched those in patients' files in 55% of cases (22 out of 40). ChatGPT options were agreed upon by 50%, 55%, and 57% of the three ocular oncology specialists. The investigation revealed significant differences between ChatGPT's responses and those of the three cancer specialists when compared to patients' files (p = 0.003, p = 0.001, and p = 0.001). ChatGPT's surgical responses matched with patient data in 18 out of 24 cases (75%), excluding enucleation cases. The decisions matched with the three ocular oncology specialists in 17/24, 18/24, and 18/24 cases, reflecting agreements of 70%, 75%, and 75%, respectively. The decisions made by ChatGPT were not significantly different from those of the three professionals in this cohort (p = 0.50, p = 0.36, and p = 0.36 for ChatGPT compared to specialists 1, 2, and 3)., Conclusion: ChatGPT exhibited a level of proficiency that was comparable to that of trained ocular oncology specialists. However, it exhibited its limitations when evaluating more complex scenarios, such as extrascleral extension or infiltration of the optic nerve, when a comprehensive evaluation of the patient is therefore necessary., Competing Interests: Declarations. Conflict of interest: No conflicting relationship exists for any author. Ethics approval: Approved by the Policlinico Universitario Agostino Gemelli Institutional Ethics Committee. Informed to consent: Not applicable. Consent to publish: No sensitive data were published., (© 2025. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: To describe a surgical technique for retinal detachment with undetected retinal breaks, which combines pars plana vitrectomy and external subretinal fluid (SRF) drainage., Methods: In this retrospective observational study, patients with a diagnosis of retinal detachment with undetected retinal breaks were enrolled. Standard three-port 25-gauge (G) core and peripheral pars plana vitrectomy was performed. Perfluorocarbon liquid was injected into the vitreous cavity to obtain posterior retinal flattening. Trans-scleral 27-G needle external drainage was performed approximately at 8 mm from limbus to drain SRF subconjunctivally. Prophylactic peripheral endolaser was performed on 360°. Sulfur hexafluoride 20% was used as tamponade., Results: In 14 of 15 patients, complete SRF drainage was obtained. In only one case, SRF did not leak out in the subconjunctival space. Neither intraoperative nor postsurgical complications were recorded. Flat retina with no SRF was observed in all samples, and no retinal detachment relapses were reported at each follow-up., Conclusion: External drainage combined with pars plana vitrectomy may represent a valid and safe option to drain SRF in retinal detachment cases with undetected retinal breaks. The advantages of the technique include the absence of dispersion of retinal pigment epithelium cells in the vitreous chamber, prompt dry retina, and low risk of postoperative retinal folds.

Purpose: To evaluate the clinicopathological characteristics and the effectiveness of post-operative high-dose-rate (HDR) interventional radiotherapy (IRT - brachytherapy) in managing advanced ocular surface squamous neoplasia (OSSN) and eyelid tumors., Methods: Nineteen patients with advanced malignancies affecting the ocular surface (stage ≥ T2) and eyelids (staging ≥ T3) were enrolled. Post-operative HDR-IRT treatment followed surgery after multidisciplinary discussion. In our series a total dose of 49 Gy was administered in 14 fractions of 3.5 Gy each, 2 doses per day. Local disease control is the study's main outcome. Death rate, total survival, disease-free survival, and toxicity are secondary outcomes., Results: Local recurrence was observed in 4 cases, 2 were conjunctival melanomas and 2 were conjunctival squamous cell carcinoma. The median OS was 56.3 months. The 12, 24 and 36 months survival rate was respectively 100.00% (IQR: 100.00% - 100.00%), 100.00% (IQR: 100.00% - 100.00%), 100.00% (IQR: 100.00% - 100.00%) respectively . The median DFS was 56.3 months. The 12, 24 and 36 months disease survival rate was respectively 85.71% (IQR: 69.21% - 100.00%), 68.57% (IQR: 42.11% - 100.00%), 68.57% (IQR: 42.11% - 100.00%) respectively. In eyelid tumors, madarosis and eyelid abnormalities are the main side effects, while in OSSNs, dry eye symptoms are frequently reported., Conclusion: Postoperative HDR-IRT has been effective in advanced eyelid cancers control. More challenging appears instead an effective treatment of advanced OSSNs, particularly conjunctival melanomas. Multicenter studies are needed to get a larger patient sample and to evaluate different radiotherapy dosages by different histologic and T types of tumors., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

Purpose: The main purpose of treatment of advanced ocular surface and periocular malignant tumors is to eradicate the tumor while trying to preserve visual function and aesthetics. Our purpose is to describe the outcome of a retrospective case series of 10 patients with advanced ocular surface and periocular tumors treated surgically in first instance and then with postoperative interventional radiotherapy (IRT/Brachiterapy)., Materials and Methods: We describe the clinicopathological features, treatments and outcome, in a retrospective case series of 10 patients with advanced tumors involving ocular surface (staging ≥ T2) and eyelids (staging ≥ T3), with involvement of periocular and/or orbit tissues. Patients were first surgically treated, most of them with incomplete excision, and then underwent a post-operative interventional radiotherapy (IRT/Brachytherapy) as an alternative to more invasive and disfiguring surgical retreatment. Tumor location, risk factors, staging, histological features, and follow-up timing were analyzed., Results: Three patients had advanced eyelid basal cell carcinomas, 2 patients were diagnosed with eyelid and conjunctival squamous cell carcinomas, 3 as sebaceous carcinomas, and 2 as primary conjunctival melanomas. The mean follow-up time from IRT to last clinical follow-up was 58.6 weeks, range 28.4-168 (median 43.65, IQR 28.9-72.9). Two patients - one with ocular surface SCC, the other with conjunctival melanoma - had a local recurrence 23.4 and 40,9 weeks after IRT, respectively. An overview of the current knowledge on adjuvant or post-operative IRT is also provided., Conclusions: IRT can be considered an effective therapeutic option to avoid more invasive surgical retreatment in advanced tumors involving eyelids and ocular surface., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: We report a case of personalized exclusive brachytherapy treatment for the management of a highly recurrent squamous cell conjunctival carcinoma with corneal invasion., Case Description: This is a case of a Caucasian 81-years-old man who presented 10 years ago to our clinic with a pink-white para-limbal mass with dilated feeder arteries and mild leukoplakia. Excisional biopsy confirmed the presence of conjunctival intraepithelial neoplasia (CIN). Successively, he underwent two 4-weeks cycles of Mytomicin C topical therapy and a second excisional surgery, due to several recurrences of the lesion. At the last relapse, the pink-white peri-limbic mass which invaded the corneal limbus, determining corneal opacification from 5- to 7-clock hours, was confirmed by anterior segment optical coherence tomography (AS-OCT). Due to resistance to MMC therapy and chronic epitheliopathy, an AS-OCT guided exclusive radiotherapy plan was set: a Rhutenium-106 CCD plaque was applied directly over the afflicted corneal surface, the corneal limbus and the neighboring sclera for 24 hours. The remission of both conjunctival and corneal malignancy was complete 2 months after surgery and no signs of recurrence were highlighted at AS-OCT analysis at the 2-year follow up., Conclusion: Brachytherapy treatment showed optimal management of both corneal and conjunctival involvement, with a free-of-disease follow-up of 24-months. This result suggests that, in specific conditions, Ru-106 brachytherapy could be an effective option of treatment even if not associated with surgical excision., Competing Interests: Disclosures The authors declare no conflict of interests. This research did not receive any specific grant from funding agencies in public, commercial or not-for-profit sectors., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Primary and metastatic leiomyosarcomas (LMS) involving the orbital region are well known to occur however, the conjunctiva represents an extremely rare site of occurrence., Methods: A 97-year-old male was referred to the Ocular Oncology Unit due to a rapidly growing painful mass (16×12×20 mm) in the nasal conjunctiva of his left eye. Wide excision followed by radiotherapy was performed., Results: Based on the microscopic features (hypercellular neoplasm composed of spindle cells with cigar shaped and blunt ended nuclei with brightly eosinophilic fibrillary cytoplasm) and immunohistochemical findings (positive staining for Vimentin, Desmin, Caldesmon, and SMA and negative staining for AE1/AE3, EMA, CD117, S100, MelanA, SOX10, HMB45, TLE1, CD99, EMA and AE1 / AE3) the final diagnosis of grade 2 leyomiosarcoma was rendered. Moreover, 'in deep' DNA sequencing (>500 genes analysis) revealed a neoplasm with high TMB: 64 muts/Mb and numerous VUS and several pathogenic/oncogenic molecular alterations, including CNV loss or gain in > 10 genes. At the last follow-up visit, residual disease was observed in the superior fornix, at the nasal limbus and the cornea. At the time of writing, after a follow-up of 2 month the patients is still alive without evidence of metastatic disease., Conclusion: An uncommon molecular finding observed in our case was the presence of TSC1 gene mutation usually associated with soft tissue and gynecological PEComas. Our finding may harbor important therapeutic implications since the inactivation of the tumor suppressor genes TSC1 and TSC2 lead to upregulation of mTOR signaling, providing the rationale for target therapy with mTOR inhibitors. Additional studies on larger series are needed to validate our findings., Competing Interests: Declaration of Competing Interest none., (Copyright © 2024 Elsevier GmbH. All rights reserved.)

The migration of silicone oil into the suprachoroidal region is a rare complication of vitrectomy. A 70-year-old man was admitted to our ocular oncology unit with a choroidal elevation in the nasal area, as well as signs of pars plana vitrectomy surgery performed about 5 years ago for retinal detachment. The patient underwent ocular ultrasound, visual field test, fluorangiography, and magnetic resonance imaging, which led to the diagnosis of silicone oil migration in the suprachoroidal space. Silicone oil may move into the suprachoroidal area, mimicking choroidal neoformation. It is important to have a comprehensive diagnosis and then set up the appropriate treatment. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:104-108.] .

Background: Periocular malignancies may be clinically different from the examples arising at other sites, with possible delayed diagnosis and greater challenges for treatment and repair. Line-field confocal optical coherence tomography (LC-OCT) is a recently developed technique characterized by an unprecedented capacity to acquire high-definition images in vertical and horizontal modes. In this study, we aimed to investigate the LC-OCT morphological features of a series of eyelid skin lesions, correlating them to histopathological findings., Methods: Patients with biopsy-proven equivocal skin lesion in the eyelid area, previously investigated by means of LC-OCT, were included in the study. Percentage overall agreement was estimated for LC-OCT and histopathological diagnosis for study cases., Results: A total of 51 patients (28 women, 23 men; mean age 66.4 years old), for a total of 51 skin lesions, were assessed. The histopathological diagnosis consisted of 30 malignant and 21 benign tumors. Different entities were characterized by peculiar findings in LC-OCT, alike to histopathological features, allowing for an accurate "in vivo" classification in almost all cases, with a diagnostic concordance with histopathology of 92.1% (47/51)., Conclusions: By integrating this new imaging technique into the assessment of suspicious tumors in this area, diagnostic accuracy may increase, improving strategies adopted in multidisciplinary meetings and patient-centered care.

Purpose: Since there is a lack of clear information regarding the benefit to combine supportive therapies (such as artificial tears) to mitomycin C (MMC) in the treatment of ocular surface neoplasia, the primary purpose of the study was to evaluate hyaluronic acid eye drops and hyaluronic acid-conjugated lactobionic acid (LACTOyal FREE) eye drops as supportive therapy., Methods: Retrospective evaluation of patients with ocular surface squamous neoplasia or conjunctival melanocytic tumor treated with MMC, who had used also artificial tears as supportive treatment. A 6-month follow-up with evaluation of subjective and objective tests for ocular surface integrity was conducted., Results: A total of 35 patients were analyzed, most of them with squamous disease (71.4%). The break-up time (BUT), Ocular Surface Disease Index (OSDI) and Schirmer test values showed a significant difference at any time point with overall population. No statistical difference was found among subgroups (Lactoyal vs No Lactoyal)., Conclusion: The use of an ancillary therapy based on hyaluronic acid allows to improve both subjective and objective ocular parameters, reducing MMC induced adverse effects. Meantime, hyaluronic acid-conjugated lactobionic acid eye drops highlighted the same advantages with a more positive trend in OSDI results., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The aim of our study was to investigate the clinical features of central serous chorioretinopathy (CSC) with autofluorescence (AF), retromode (RM), and enface imaging. This retrospective study was conducted at Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome (Italy), between September and December 2022. Each patient underwent a complete ophthalmological examination, which included optical coherence tomography (OCT), enface image analysis, AF, and RM imaging. We further evaluated the presence and area of extension of serous retinal detachment and retinal pigment epithelium (RPE) atrophy through AF, RM, and enface imaging. We included 32 eyes from 27 patients (mean age: 52.7 ± 13.3 years). The median AF area was 19.5 mm 2 (IQR 6.1-29.3), while the median RM area was 12.3 mm 2 (IQR 8.1-30.8), and the median enface area was 9.3 mm 2 (IQR 4.8-18.6). RPE atrophy was diagnosed in 26 cases (81.3%) with RM imaging and in 75% of cases with AF. No difference emerged between AF and RM analysis in the detection of central serous detachment in CSC. However, RM imaging showed a high specificity (91.7%) and negative predictive value (84.6%) to detect RPE changes when compared to the AF standard-of-care technique. Thus, RM imaging could be considered an adjunctive imaging method in CSC.

Uveal melanoma is the most common ocular tumor with frequent metastatic spread to the liver. Immune checkpoint inhibitors have demonstrated poor results in this disease. The addition of hepatic radiotherapy to anti-PD-1 could enhance the sensitivity to immunotherapy. In this study, patients treated with pembrolizumab and who have undergone hepatic radiotherapy have been retrospectively evaluated. Twenty-two patients have been considered. Six patients (27.3%) achieved a partial response and 3 (13.6%) a stable disease. Disease control rate was 40.9%. Thirteen patients (59.1%) had progression as best response. The median PFS was 4.8 months and 6 months PFS rate 45.4%. The median OS was 21.2 months, while 1 year OS rate was 72.7%. Longer survival was observed in patients who achieved a partial response on irradiated metastases (HR 0.23, 95% CI 0.06-0.83) or progressed after 6 months (HR 0.12-95% CI 0.03-0.44). No radiotherapy-related or grade 3-4 adverse events were reported. This study demonstrates that the addition of hepatic radiotherapy to anti-PD-1 treatment can be a valid option for the treatment of metastatic uveal melanoma, particularly for HLA A 02:01 negative patients. Prospective studies should be conducted to confirm these data.

Introduction: The aim of this study was to explore the early efficacy and safety of treatment with intravitreal injections (IVIs) of brolucizumab in patients presenting with neovascular age-related macular degeneration (nAMD) in a real-world setting., Methods: This retrospective study included 194 eyes of 180 patients with nAMD treated with standard 6-mg IVIs of brolucizumab in our clinic between March 11, 2021, and June 15, 2022. Both treatment-naive (33 eyes) and switch therapy patients (161 eyes) were included in the study. Best corrected visual acuity (BCVA), central subfield thickness (CST), retinal fluid distribution (classified as intraretinal, subretinal, under the pigmented epithelium), treatment intervals, and adverse event rates were collected for analysis., Results: Average follow-up time was 37.2 ± 16.6 weeks. Mean baseline BCVAs were 38.1 ± 4.5 and 41.9 ± 6.7 letters in the treatment-naive and switch therapy groups, with a final gain of 16.0 ± 4.9 (p < 0.0001) and 10.7 ± 5.9 (p < 0.0001) letters in the two groups, respectively. Throughout the study period, CST significantly decreased in both treatment naïve (from 352.0 ± 129.4 to 284.2 ± 93.8 µm; p = 0.0015) and switch therapy (from 369.9 ± 140.5 to 307.4 ± 123.5 µm; p < 0.0001). Significant fluid control rates were achieved at the end of the study period (45% and 27% eyes were completely free of fluid in naïve and switch groups, respectively). Five eyes (2.6%) developed adverse events with different grades of intraocular inflammation and visual outcomes., Conclusion: Brolucizumab IVI showed very good anatomical and functional outcomes in both naive and switch patients in this real-world experience. Nevertheless, even showing a favorable risk/benefit profile, clinicians and patients should be aware of the possibility of a small rate of severe complications., (© 2023 S. Karger AG, Basel.)

Purpose: The complexity of multimodal approaches in cancer management has lately led to the establishment of multidisciplinary tumor boards (MDTBs) to define targeted, patient-centered treatment strategies. However, few data are available regarding the application of this approach in Ocular Oncology. Hereby, the Authors analyze the implementation and outcomes of a trained MDTB in a tertiary ocular oncology referral center., Methods: A retrospective descriptive analysis of MDTB meetings discussing patients with ocular and periocular cancers, over a 12-months period, was carried out. Data were grouped by main site involved, topics discussed and final clinical decisions therefore taken. Meetings were held by a constant 'Core team' or - when required - by a broader 'Extended team'., Results: During the observational period 86 cases were discussed. In 27 patients ocular surface tissues were involved (31%), in 25 patients orbital tissues (29%), in 22 patients eyelids (26%), and in 12 patients intraocular tissues (14%). In 13 cases (15%) naïve or referred new patients, in 34 cases (40%) imaging or histopathologic reports and in 39 cases (45%) treatment plans were discussed. Regarding final decisions, a treatment plan was scheduled in 47 cases (55%) and a diagnostic ascertainment was required in 27 patients (31%); locally advanced and/or systemic diseases were referred or teamed up with other specialists in 12 cases (14%)., Conclusions: Ocular Oncology multidisciplinary team, by sharing expertise of different specialists, ensures a comprehensive evaluation of patients improving the accuracy of diagnosis and staging upon which planning a proper treatment. Further studies are needed to assess if this approach may also improve the outcomes and prognosis of patients., (© 2022. The Author(s).)

Purpose: To evaluate the effect of photodynamic therapy (PDT) as adjuvant treatment, after brachytherapy, in posterior amelanotic choroidal melanomas., Methods: Six patients with posterior amelanotic choroidal melanoma underwent brachytherapy treatment. Tumour response was assessed by fundus examination, fundus photography and A-B scan ultrasonography. The residual tumours were treated with adjuvant PDT performed with infusion of verteporfin intravenously at 6 mg/m 2 body surface area. Five minutes after infusion, a 689 nm laser was applied with a light dose of 100 J/cm 2 over an interval of 166 s., Results: At a median follow-up after brachytherapy of 17.5 months (IQR 16.2-22.5, range 5-42 months), tumours showed a partial reduction of tumour thickness (22.5% as compared to baseline value) and persistent low internal reflectivity at A-B scan ultrasonography. Supplementary photodynamic treatment resulted in complete resolution of the lesion with marked decrease of elevation . Mean decrease in thickness after PDT was 49.9% with respect to previous brachytherapy treatment 22.5% (p = 0.007). The results was achieved within a median period of 4 months (range 2-4 months) after PDT, and there has been no recurrence after a median follow-up of 84.7 months ± 18.7 (range 59 to 107 months)., Conclusion: Combined treatment of brachytherapy and adjuvant PDT in amelanotic uveal melanoma seems to be favourable with regard to complete and rapid tumour regression.

Primary malignant epithelial tumours arising from accessory lacrimal glands (ALGs) are extremely rare, with only few cases reported in literature. They generally appear as gradually increasing masses of the upper or the lower eyelid. Only one case of primary adenocarcinoma or adenocarcinoma not otherwise specified (ACNOS) from ALGs has been reported in literature. Herein, we describe a case of ACNOS arising from ALGs with an atypical clinical presentation and review prior cases of ALGs epithelial malignancies reported in the literature. A 78-year-old man referred to our Ocular Oncology Unit for adjuvant therapy after the excision of a conjunctival tumour of the left eye with a histological diagnosis of squamous cell carcinoma. He underwent topical chemotherapy with MMC and during follow up he presented a multinodular iris mass in his left eye. The MRI of the orbit showed an ocular mass infiltrating orbital soft tissues of the inferior palpebral region with an involvement of the corresponding zygomatic cutis. We performed orbital exenteration and histological studies revealed an epithelial neoplasm with a solido-glandular growth pattern with lumens containing an eosinophilic material positive for PAS and PAS-D. The immunohistochemical findings confirmed the diagnosis of adenocarcinoma NOS from ALGs. Although ALGs epithelial malignancies are extremely uncommon, they should be considered in the differential diagnosis of ocular tumours. A vigilant approach towards these entities is required, since they can be clinically insidious and locally aggressive.

Purpose: To report a case of macular choroidal osteoma treated with photodynamic therapy., Observations: A 34-years old woman with decreased visual acuity in her left eye came to our observation for assessment of an amelanotic choroidal tumor in the left eye. On the basis of ophthalmoscopic and echographic features the tumor was diagnosed as choroidal osteoma. Imaging examination revealed subretinal fluid involving the foveal area associated with alterations of outer neuroepithelial layers and photoreceptors without evidence of choroidal neovascularization. Foveal sparing standard fluence rate photodynamic therapy was performed. After treatment, subretinal fluid reabsorption and visual acuity recovery was noted with progressive restoration of foveal architecture. Due to the relapse of fluid and visual impairment, 1 year after treatment, a second PDT session was made using the same parameters and protocol of treatment. Despite a complete subretinal fluid reabsorption and visual acuity recovery the second treatment was complicated by the development of subretinal fibrosis., Conclusions: PDT is effective to induce subretinal fluid reabsorption and visual recovery in choroidal osteoma located in the macular area. However, the risk of possible complications related to the treatment have to be considered.

Purpose: Orbital pseudotumor (or pseudotumor orbitae) is a benign entity, also known as idiopathic orbital inflammatory syndrome (IOIS), which encompasses a wide range of non-tumoral, space occupying lesions of the orbit. In selected cases of refractory disease or presence of side effects or even comorbidities that limit systemic therapies, radiotherapy could play a role in the management because it has been demonstrated that irradiation is effective in suppressing the inflammatory process., Methods: A systematic review of the literature about the main scientific databases was launched and the time interval included all published articles present in the databases from their inception until September 2020., Results: We were able to identify 19 studies eligible for inclusion in this review from 1978 to 2018. Overall the data of 241 patients were collected and are presented in this systematic review. The response rate varied between 74% and 100% with a median recurrence rate of 10%. The median total dose was 20 Gy whereas the mean total dose was 21 Gy with a range from 4 to 36 Gy. Regarding the fractionation, 2 Gy/fraction daily was the most widely used., Conclusions: Radiotherapy seemed to achieve good response rates however, in most of the studies, inclusion criteria and outcome parameters are not uniform and therefore the results are difficult to compare. Often important parameters such as chronic pain and permanent functional deficits are not assessed in the outcome. Therefore, prospective studies, with good cohort characteristics and a clear definition of the outcome, are required.

Metastatic uveal melanoma (UM) is a poor prognosis malignancy. Immunotherapy is commonly employed, despite the low activity, considering the lack of other effective systemic treatments. In this study, the prognostic and predictive role of soluble immune checkpoints and inflammatory cytokines/chemokines in 22 metastatic UM patients was evaluated. Baseline levels of these molecules were assessed, as well as their changes during anti-PD-1 therapy. The correlation between soluble immune checkpoints/cytokines/chemokines and survival was analyzed. A comparison between circulating immune profile of metastatic cutaneous melanoma (CM), for which immunotherapy is a mainstay of treatment, and UM during anti-PD-1 therapy was also performed. Three immune molecules resulted significantly higher in metastatic UM patients with survival <6 months versus patients with survival ≥6 months: IL-8, HVEM and IDO activity. Considering these three molecules, we obtained a baseline score able to predict patients' survival. The same three molecules, together with soluble(s) CD137, sGITR and sCD27, resulted significantly lower in patients with survival >30 months. We also observed an increase of sCD137, sCD28, sPD-1, sPD-L2 sLAG3, sCD80 and sTim3 during anti-PD-1 treatment, as well as IDO activity, IP-10 and CCL2. Several of these molecules were significantly higher in UM compared to CM patients during anti-PD-1 therapy. The analysis of circulating immune molecules allows to identify patients with poor prognosis despite immunotherapy and patients with long survival treated with an anti-PD-1 agent. The different serum concentration of these molecules during anti-PD-1 therapy between UM and CM reflects the different efficacy of immune checkpoint inhibitors.

Uveal melanoma (UM) is the most common intra-ocular tumor in Caucasian adults and may derive from melanocytes. Historically, the first therapeutic approach to treat UM has been surgical removal of the eye, with obvious consequences in terms of function, cosmesis, and psychological impact on patients. Collaborative Ocular Melanoma Study (COMS) introduced the first uniform approach to perform interventional radiotherapy (IRT - brachytherapy) procedure in a standardized way that allowed to demonstrate equivalence of IRT with enucleation in terms of overall survival. Since this milestone, several international guidelines have been issued with regard to several technical aspects of this procedure, which has become the mainstream therapy worldwide. However, nowadays, there is still a lack of consensus about the criteria to definitively assess UM response after IRT. We present a collection of paradigmatic cases treated in our institution, and then discuss in detail the latest available evidence on the topic., Competing Interests: The authors report no conflict of interest., (Copyright © 2022 Termedia.)

Purpose: To analyze the clinical characteristics and long-term follow-up of patients with advanced ocular surface squamous cell carcinoma (OSSC) involving periocular tissues and/or orbit. Primary outcomes were overall survival (OS), disease-free survival (DFS), and overall recurrence rate (RR). Secondary outcomes were a correlation between primary outcomes and tumor location, American Joint Committee on Cancer Classification (AJCC) staging system, histological results, surgical margins, and type of treatment., Study Design: a retrospective case series., Methods: The medical records of patients affected by OSSC involving periocular tissues and/or orbit referring, from 01/2011 to 01/2020, to our tertiary referral center were reviewed., Results: Thirty-six eyes of 36 patients were included. The mean age was 68.2 years; 18 (50%) patients were males. The mean follow-up was 40 months. The RR was 64%. The OS at 12, 24, 36, and 60 months was respectively 97.1%, 92.7%, 92.7%, and 92.7%. The DFS at 12, 24, 36, and 60 months was respectively 62.9%, 50.8%, 41.6%, and 29.7%. Multicentric disease (p = 0.0039), inferior tarsus localization (p = 0.0428), histological diagnosis of high-risk SSCs (p = 0.0264), positive surgical margins (p = 0.0434), and excisional biopsy (EB) alone (p = 0.0005) were associated with an increased risk of recurrence. A shorter OS was observed in patients who underwent EB alone (p = 0.0049)., Conclusion: OSCC involving periocular tissues and/or orbit is an aggressive disease with a high recurrence rate. Multicentric disease, positive surgical margins, inferior tarsus localization, and surgery without adjuvant therapies are strong predictors of recurrence and are the main factors affecting prognosis., (© 2021. The Author(s).)

Introduction and Purpose: Choroidal metastases (CM) are the most common intraocular malignancies. With longer survival rates for cancer patients, CM will be increasingly encountered. We evaluated clinical and ultrasonographic (US) characteristics of CM in order to identify diagnostic biomarkers that correlate with the primary tumor site., Methods: The medical records of all patients with CM evaluated at the Ocular Oncology Unit between February 2010 and March 2020 were analyzed., Results: 82 eyes of 70 patients were included. The primary cancer site was lung in 26 patients (37%), breast in 23 (33%), kidney in 9 (13%), gastrointestinal in 5 (7%), thyroid in 5 (7%), parathyroids and prostate respectively in 2 (3%). Fifty-five patients (78%) had other systemic metastases at the time of ocular diagnosis. Ten (14%) patients had no history of primary cancer. Bilateral CM were found in 20 patients (29%); fifty-six eyes (68%) had a single CM. The epicenter of CM was predominantly macula (43 eyes, 52%). The mean thickness was 4,1 mm (range 1,8-12,3). US structure was inhomogeneous in 67 eyes (82%). Reflectivity was mainly medium (39%) and medium-low (39%). In particular, CM from lung cancer showed lower reflectivity than those from the breast (p = 0,02). CM deriving from lung cancer were typically dome-shaped, whereas CM originating from breast were characteristically plateau shaped (p = 0,02). Seventy-four (91%) eyes presented fluid on optical coherence tomography., Conclusion: We significatively found that CM from lung cancer generally appear dome-shaped with medium-low internal reflectivity, whereas those from breast cancer typically present a plateau appearance and higher internal reflectivity. Though it is hard to identify the site of the primary tumor relying exclusively on clinical and US aspects, morphology and internal reflectivity can be considered as diagnostic biomarkers. Thus, the origin of the primary tumor can be suspected by integrating a constellation of findings., Competing Interests: The authors have declared that no competing interests exist.

Immunotherapy can be used for cutaneous, mucosal, uveal and conjunctival melanoma. Nevertheless, we cannot expect the same benefit from checkpoint inhibitors for all the types of melanoma. The different biological features can explain the variable efficacy. The main results obtained with immune checkpoint inhibitors in the various types of melanoma were reviewed.

Purpose: Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography., Methods: Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography., Results: On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion., Conclusion: Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.

Aim: The aim of this study was to detect clinical factors predictive of loss of visual acuity after treatment in order to develop a predictive model to help identify patients at risk of visual loss., Patients and Methods: This was a retrospective review of patients who underwent interventional radiotherapy (brachytherapy) with 106 Ru plaque for primary uveal melanoma. A predictive nomogram for visual acuity loss at 3 years from treatment was developed., Results: A total of 152 patients were selected for the study. The actuarial probability of conservation of 20/40 vision or better was 0.74 at 1 year, 0.59 at 3 years, and 0.54 at 5 years after treatment. Factors positively correlated with loss of visual acuity included: age at start of treatment (p=0.004) and longitudinal basal diameter (p=0.057), while distance of the posterior margin of the tumor from the foveola was inversely correlated (p=0.0007)., Conclusion: We identified risk factors affecting visual function and developed a predictive model and decision support tool (AVATAR nomogram)., (Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

No standard treatment has been established for metastatic uveal melanoma (mUM). Immunotherapy is commonly used for this disease even though UM has not been included in phase III clinical trials with checkpoint inhibitors. Unfortunately, only a minority of patients obtain a clinical benefit with immunotherapy. The immunological features of mUM were reviewed in order to understand if immunotherapy could still play a role for this disease.

Background: No standard treatment has been defined for metastatic uveal melanoma (mUM). Although clinical trials testing Nivolumab/Pembrolizumab for cutaneous melanoma did not include mUM, anti PD-1 agents are commonly used for this disease., Patients and Methods: In this prospective observational cohort single arm study, we investigated efficacy and safety of Pembrolizumab as first-line therapy for mUM. The efficacy was evaluated in terms of progression-free survival (PFS), response rate and overall survival (OS). Toxicity was also assessed., Results: Seventeen patients were enrolled. A median of 8 cycles were administered (range 2-28). Two patients achieved partial response (11.7%), 6 a disease stabilization (35.3%), whereas 9 (53%) had a progression. No complete response was observed. PFS of the overall population was 3.8 months. PFS was 9.7 months for patients with an interval higher than 5 years from diagnosis of primary tumor to metastatic disease and 2.6 months for patients with an interval lower than 5 years [p = 0.039, HR 0.2865 (95% CI 0.0869-0.9443)]. Median OS was not reached. The two responding patients were still on treatment with Pembrolizumab at the time of data analysis. Survival was 12.8 months for patients with clinical benefit, while OS for progressive patients was 3.1 months. PD-L1 expression and genomic abnormalities predictive of relapse after diagnosis of primary tumor were not associated with PFS. Toxicity was mild, without grade 3-4 side effects., Conclusions: The efficacy of Pembrolizumab does not seem particularly different when compared to other agents for mUM, but responding patients had a remarkable disease control.

Conjunctival melanoma is a rare primary ocular tumor. So far, no standard treatment exists for metastatic disease. Similarly to cutaneous melanoma, up to 50% of conjunctival melanomas harbor BRAF mutations. The most common is represented by V600E. Combined therapy with BRAF and MEK inhibitors is approved for BRAF mutant cutaneous metastatic melanomas. Herein, we report a case of a 70-years old patient with a metastatic conjunctival melanoma harboring V600E BRAF mutation successfully treated with dabrafenib and trametinib.

Purpose: To report the results of a patient's tailored therapeutic approach using a second course of interventional radiotherapy (brachytherapy) in patients with locally recurrent uveal melanoma., Material and Methods: Patients who had already undergone ocular brachytherapy treated at our IOC (Interventional Oncology Center) were considered. Five patients who has received a second course of treatment with a plaque after local recurrences were included in our study. Re-irradiation was performed with Ruthenium-106 (prescribed dose to the apex 100 Gy) or with Iodine-125 plaques (prescribed dose to the apex 85 Gy). Moreover, a systematic literature search was conducted through three electronic databases, including Medline/PubMed, Scopus, and Embase., Results: All patients were initially treated with Ruthenium-106 plaque; the re-irradiation was performed with Ruthenium-106 plaque in three cases and with Iodine in two cases. Mean time between the first and the second plaque was 56.8 months (range, 25-93 months). Local tumor control rate was 100%, no patient underwent secondary enucleation owing to re-treatment failure. Distant metastasis occurred in 1 patient after 6 months from re-treatment. After a median follow-up of 44.2 months (range, 26-65 months) from re-treatment, all patients experienced worsening of the visual acuity (median visual acuity was 0.42 at time of recurrence and decline to 0.24 at the most recent follow-up); cataract occurred in two cases, no patient developed scleral necrosis. We considered 2 papers for a systematic review., Conclusions: In selected cases, especially in presence of marginal local recurrence, a personalized re-treatment strategy with a plaque may offer high probability of tumor control and organ preservation but worsening of visual acuity., Competing Interests: Authors report no conflict of interest.

Purpose: To analyze the relationship between swelling of the arcuate nerve fiber layer (SANFL) and long-term decrease of retinal nerve fiber layer thickness after internal limiting membrane peeling for idiopathic epiretinal membrane, and to investigate if SANFL is related to a mechanical surgical damage., Methods: Prospective, interventional consecutive case series of 46 eyes that underwent combined epiretinal membrane/internal limiting membrane peeling for idiopathic epiretinal membrane. Infrared, blue autofluorescence, color fundus imaging and measurement of retinal nerve fiber layer thickness in six peripapillary sectors by spectral-domain optical coherence tomography were performed preoperatively and at 2 weeks, 1, 3, 6, and 12 months after surgery. The presence of SANFL was checked postoperatively on infrared and blue autofluorescence fundus imaging, and the extent of each SANFL was measured on infrared fundus images., Results: Areas of SANFL were identified in 39 eyes (84.8%) at 2-week follow-up. Retinal nerve fiber layer thickness significantly decreased in the temporal sectors at 1, 6, and 12 months (P < 0.0001). The linear extent of SANFL was significantly correlated with the percentage of reduction in retinal nerve fiber layer thickness in the temporal (R = 0.45; P < 0.0001) and infero-temporal (R = 0.23; P = 0.0008) sectors at 12 months of follow-up. Correspondence between sites of surgical grasping and the points of origin of SANFL was demonstrated on blue autofluorescence fundus images superimposed on intraoperative surgical frames., Conclusion: Early postoperative SANFL is correlated with late focal retinal nerve fiber layer thinning in the temporal sectors. Intraoperative surgical grasping seems to be a leading factor for the onset of SANFL.

Purpose: To report the case of a 13-year-old girl with torpedo maculopathy, evaluated with multimodal morpho-functional retinal imaging, including fundus photography, infra-red and blue fundus autofluorescence, swept-source optical coherence tomography (OCT), en face OCT, OCT angiography and microperimetry (MP)., Observations: On fundus examination, a torpedo-like hypopigmented lesion was observed temporal to the fovea in the left eye. OCT showed disruption of outer retinal layers and the presence of a subretinal cleft. On OCTA, a diffuse attenuation of signal from choriocapillaris was observed along the lesion. Functional analysis with MP revealed a reduction of retinal sensitivity over the lesion., Conclusions: and importance: On OCTA, torpedo maculopathy is characterized by vascular alterations of the choriocapillaris along the lesion.

Background: Traditionally, surgical excision has been the treatment of choice for ocular surface squamous neoplasia (OSSN). Recurrences after surgery are high. To reduce the risk of recurrence, adjuvant therapies have been increasingly used., Purpose: We compared recurrences and complications of 3 forms of treatment for OSSN: surgical excision (group A), surgical excision plus adjuvant topical mitomycin C (MMC) (group B), and surgical excision plus subconjunctival interferon-α-2b (IFN-α-2b) (group C)., Methods: A retrospective comparative study was conducted between January 2006 and March 2016 at the Ocular Oncology Service of the Catholic University of Rome. Seventy-nine patients with a confirmed histological diagnosis of OSSN were included: 43 were treated with surgical excision (group A), 16 underwent surgical excision plus topical MMC (group B), and 20 underwent surgical excision plus adjuvant subconjunctival IFN-α-2b (group C)., Results: The recurrences were different in the 3 groups. Thirty-one recurrences (72%) were seen in group A, 5 (31%) were found in group B, and 3 (15%) were seen in group C. Eight (50%) patients who received MMC 0.02% complained of ocular discomfort, 10 (62.5%) presented conjunctival hyperemia, while conjunctival chemosis and corneal epitheliopathy were noticed in 2 (13%) and 2 (13%) patients, respectively. All patients treated with subconjunctival IFN-α-2b reported flu-like symptoms. Two patients (10%) complained of ocular discomfort., Conclusions: Our study revealed that OSSN is not always manageable with simple excision. Adjuvant chemotherapy is strongly advisable after surgery to reduce recurrences. Interferon injections and MMC drops are effective in preventing recurrences and should be administered after surgery.

Although introduced for the treatment of maculopathies, photodynamic therapy (PDT) is now largely used in some eye cancers treatment. The selective tissue damage with PDT is achieved by sequestration of the photosensitizer in the target tissue and focal activation of the photosensitizer by low energy directed light. In this way, it is possible to achieve the destruction of the tumor tissue by safeguarding the surrounding healthy structures. Our study describes the clinical uses and efficacy of photodynamic therapy in ocular oncology., Competing Interests: The authors declare no conflict of interest.

Purpose: The purpose of this study was to study the incidence and factors influencing retinal displacement in eyes treated for rhegmatogenous retinal detachment (RRD) with pars plana vitrectomy (PPV) and gas or silicone oil., Methods: This was a prospective observational case series. One hundred twenty-five eyes with macula-off RRD from 125 patients underwent 25-gauge PPV at two vitreoretinal institutional practices. Eyes without proliferative vitreoretinopathy (PVR) or PVR grade A were tamponated with sulfur hexafluoride (SF6) gas, whereas eyes with PVR grade B received 1000 centistokes silicone oil (SO). The patients postured face-down immediately after surgery. Blue-fundus autofluorescence (B-FAF) pictures were obtained at each follow-up examination. Main outcome measures were incidence and direction of retinal displacement., Results: Ninety-seven eyes (77.6%) were tamponated with SF6 and 28 eyes (22.4%) with SO. After retinal reattachment, displacement was observed in 44 of 125 (35.2%) eyes (40 eyes in the SF6 group and 4 eyes in the SO group, respectively). The type of tamponade, specifically gas, was the only significant predictor of retinal displacement (P = 0.007). The displacement was downward in 39 (88.6%) eyes (36 tamponated with SF6 and 3 with SO) and upward in 5 (11.4%) eyes (4 tamponated with SF6 and 1 with SO)., Conclusions: Displacement of the retina after repair of macula-off RRD with PPV is observed using either SF6 gas or SO. Downward and upward displacements may occur with both tamponades, but downward dislocation is more common. Of the factors potentially implicated in favoring displacement that were studied, only the type of tamponade, specifically the gas, was significant.

Purpose: To verify the efficacy of 25-gauge pars plana vitrectomy (PPV) for the management of posteriorly dislocated lens material after complicated cataract extraction and to determine in what patients this approach offers the optimal benefit in terms of efficacy and safety, considering the amount of retained nuclear material and the duration of surgery., Methods: Forty eyes of 40 patients with retained lens fragments undergoing early (within 1 week) or late (within >1 week) 25-gauge PPV were retrospectively reviewed. The amount of dislocated nuclear material was graded by the surgeon intraoperatively, and the patients were divided into two groups according to the nuclear grading: group A (≤50% dropped nucleus) and group B (>50% dropped nucleus). The presence of brunescent nuclear pieces was considered. The outcomes measured included best-corrected visual acuity (BCVA) and postoperative complications such as retinal detachment, cystoid macular edema (CME) and postoperative ocular hypertension or hypotonia., Results: The patients had a mean age of 78 years. The mean preoperative logarithm of the minimum angle of resolution (logMAR) BCVA was 0.57 ± 0.24 (20/80). A significant positive correlation was found between nuclear material grade and PPV duration (R2 = 0.81, p < 0.0001). None of the patients had dislocation of brunescent nuclear pieces. On postoperative day 1, the mean postoperative intraocular pressure was 16.75 ± 2.7 mm Hg, with no case of ocular hypotonia. At 6 months of follow-up, the mean logMAR BCVA improved to 0.23 ± 0.3 (20/32). Retinal detachment developed in 4 patients (10%), occurring only in patients of group B (p < 0.002). Four patients with late PPV developed postoperative CME, with no case of CME among patients with early vitrectomy (p = 0.014)., Conclusion: Removal of dislocated lens fragments after complicated cataract surgery can be effectively managed with 25-gauge PPV, although it appears to be most efficient for cases with a limited amount of dislocated lens material. In consideration of the higher rate of retinal detachment observed in cases of prolonged PPV time, the expected duration of surgery should be taken into account when choosing the best surgical approach. Visual outcomes are not affected by the timing of PPV, whereas early vitrectomy seems to prevent the onset of inflammatory macular edema., (© 2015 S. Karger AG, Basel.)

Purpose: The aim of this study was to evaluate a novel surgical combination of Descemet stripping automated endothelial keratoplasty (DSAEK) and deep sclerectomy (DS) for the management of concomitant corneal endothelial decompensation and uncontrolled glaucoma., Methods: This retrospective case series noncomparative study included 9 eyes of 6 consecutive patients with coexistence of corneal edema resulting from Fuchs dystrophy or pseudoexfoliation keratopathy and medically uncompensated glaucoma; these patients underwent combined DSAEK and DS with mitomycin C and an absorbable collagen implant. Corneal graft clarity, endothelial cell density, visual acuity, intraocular pressure (IOP), and identification of complications were assessed over a 2-year follow-up., Results: All eyes obtained graft clarity throughout the follow-up, with a final average endothelial cell decrease of -36% from baseline, and showed improved vision and good IOP control without hypotensive therapy. Measured at 3 and 24 months postoperation, the mean visual acuity improvement was 154% and 372% and IOP decrease was 51.1% and 46.4%, respectively. Two anterior segment complications occurred in 2 (22%) patients' eyes. This consisted of a graft dislocation and a modest IOP elevation, treated successfully., Conclusions: Combined DSAEK and DS was longitudinally associated with good corneal graft survival and IOP control, with few complications. These findings suggest that this surgical approach is a viable option for patients with coexisting glaucoma and corneal endothelial dysfunction. Our study should stimulate a multicenter, randomized, controlled trial of our technique.

Purpose: Acute exudative polymorphous vitelliform maculopathy is a rare retinal disease characterized by bilateral serous macular detachment and subretinal accumulation of yellowish deposits resembling Best dystrophy lesions. Corticosteroid systemic therapy has been used empirically in the attempt to treat this retinal disorder with mixed results. Thus, the benefit of corticosteroid remains undetermined. To our knowledge, we report the first case of acute exudative polymorphous vitelliform maculopathy (AEPVM) treated in one eye with intravitreal dexamethasone implant (Ozurdex; Allergan, Inc., Irvine, California, USA)., Methods: A 28-year-old man with AEPVM underwent intravitreal dexamethasone implantation in the left eye., Results: Compared with the fellow eye, intravitreal dexamethasone implant did not significantly modify the clinical course of the disease. No implant-related complication was experienced during follow-up., Conclusions: The lack of response to intravitreal dexamethasone seems to suggest that corticosteroids may be ineffective for the treatment of AEPVM.

Purpose: To evaluate whether standardized uptake value (SUV) and/or metabolic rate of glucose (MRglu) are different among epithelioid, mixed, and spindle cell uveal melanomas, as well as between low and high risk melanomas; to correlate ultrasonographic data and metabolic parameters with histopathological features; and to assess the role of (18)F-FDG PET/CT for evaluating prognosis., Methods: Of 34 eligible patients prospectively enrolled with clinical suspicion of medium/large uveal melanoma, 26 (15 men, mean age 62.8 ± 11.8 years) were evaluated. All patients underwent metastatic work-up, 3-D dynamic brain and whole-body (18)F-FDG PET/CT, and surgery., Results: Of the 26 ocular lesions, 23 showed (18)F-FDG uptake, with a sensitivity of 88 %. MRglu was significantly higher in the epithelioid cell melanomas than in the spindle cell melanomas, as well as in high-risk lesions than in low-risk lesions (p = 0.01, p = 0.02, respectively). SUV and MRglu were correlated with histopathological features while ultrasonographic data were not., Conclusion: MRglu is useful for distinguishing the different cell types in uveal melanoma, as well as high-risk from low-risk lesions, while SUV is not. MRglu provides a more accurate evaluation of glucose consumption, whereas SUV provides only an estimation. In addition, the metabolic parameters correlate with histopathological features, well also reflecting cellular behaviour in ocular malignancy. A longer follow-up is needed to assess the role of (18)F-FDG in evaluating prognosis.

Objective: To report the long-term therapeutic results for patients with conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma who were treated with intralesional injections of interferon-α (IFN-α)., Design: Prospective, nonrandomized, interventional case series., Participants: Twenty eyes of 16 patients with histologically proven conjunctival MALT lymphoma in the absence of systemic disease., Methods: Patients were given 1,500,000 international units (IU) of IFN-α (Roferon-A; Roche s.p.a., Milano, Italy) subconjunctivally inside the lesion 3 times weekly for 4 weeks. If there was even a minimal response, a further cycle of 1,000,000 IU 3 times weekly for 4 weeks was administered., Main Outcome Measures: Patients were followed up clinically using slit-lamp examination to determine evidence of tumor disappearance or recurrence. In 10 eyes, an incisional biopsy was performed 6 months after therapy to verify the histologic absence of the lesion., Results: A complete response was obtained in 15 eyes (75%) at the end of first cycle treatment, and in 5 eyes (25%) after further cycles. Seventeen eyes (85%) showed no local recurrence after a median follow-up of 65 months (range, 15-136 months). Three eyes (15%) demonstrated recurrence at variable points after treatment. One patient with stage IIA lymphoma exhibited systemic lymphoma progression., Conclusions: Local immunotherapy with IFN-α seems to be an effective and lasting treatment method and provides an alternative to radiotherapy for conjunctival MALT lymphomas. Very few transient side effects were detected., (Copyright © 2012 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To report a case of a lymphoepithelioma-like carcinoma (LELC), which is a variant of carcinoma most commonly involving the nasopharynx and rarely occurring in a variety of sites outside its typical location., Methods: We present the case of a 79-year-old Caucasian man with LELC of the lacrimal gland undergoing surgical resection of the mass., Results: The patient had remained disease-free for 2 years, and then he developed homolateral metastases in latero-cervical and parotid lymph nodes, detected by computed tomography and confirmed by fine-needle aspiration biopsy. He did not receive any surgical treatment, radiotherapy, or chemotherapy because of the advanced stage of the disease and poor health condition., Conclusions: This case report suggests that a diagnosis of lacrimal gland LELC makes combined early and radical therapy necessary.