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ABSTRACT Purpose: To describe cellular alterations detected by impression cytology of the ocular surface in patients with xeroderma pigmentosum. The secondary objective was to assess the reliability of impression cytology in diagnosing ocular surface squamous neoplasia. Methods: Patients with xeroderma pigmentosum underwent a single-day complete ophthalmological examination and impression cytology for ocular surface evaluation using 13 mm diameter mixed cellulose esters membrane filters and combined staining with Periodic Acid Schiff, Hematoxylin and Eosin, and Papanicolaou stains followed by microscopic analysis. The cytological findings were correlated with the clinical diagnosis. The impression cytology findings at baseline and one-year follow-up were correlated with the clinical course (no tumor, treated tumor, residual tumor recurrent tumor, new tumor). Results: Of the 42 patients examined, impression cytology was performed in 62 eyes of 34 participants (65% females). The mean age of patients was 29.6 ± 17 years (range 7-62). Fifteen eyes had a clinical diagnosis of ocular surface squamous neoplasia. Impression cytology showed goblet cells (47, 75%), inflammatory cells (12, 19%), keratinization (5, 8%), and squamous metaplasia (30, 48%). Impression cytology was positive for atypical cells in 18 patients (12 with and 6 without ocular surface squamous neoplasia). The sensitivity, specificity, positive predictive value, and negative predictive value of impression cytology (at baseline) for diagnosis of ocular surface squamous neoplasia were 80%, 87%, 67%, and 93%, respectively, using clinical diagnosis of ocular surface squamous neoplasia as the reference standard. Conclusion: Impression cytology has a moderate positive predictive value for the diagnosis of ocular surface squamous neoplasia in patients with xeroderma pigmentosum. However, the lack of detection of atypical cells on impression cytology has a high negative predictive value for ocular surface squamous neoplasia. Integration of impression cytology in the long-term management of high-risk patients, such as patients with xeroderma pigmentosum, can avoid unnecessary diagnostic biopsies.

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ABSTRACT We present a rare case of primary caruncle basal cell carcinoma (BCC), a condition with limited occurrences. Our patient, an 80-year-old woman without prior ocular pathological history, presented a 2x2mm pedunculated blackish nodular lesion on the caruncle of her left eye, without local conjunctival or cutaneous involvement. Histological analysis following complete excision confirmed the presence of basal cell carcinoma within the caruncle. Over a span of 30 months, no recurrence has been observed. While scant cases are documented in the literature, we conducted a review of these instances. Despite its infrequent manifestation, this condition should be taken into account when evaluating caruncular tumors, given its tendency to invade the orbit. Complete excision with free surgical margins is the treatment of choice, and adjuvant radiotherapy or chemotherapy might be considered.

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Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic type of UMs in order to verify the role of diffusion-weighted magnetic resonance imaging (DWI) as a noninvasive prognostic marker. A total of 26 patients with UMs who had undergone MRI and subsequent primary enucleation were retrospectively selected. The ADC of the tumor was compared with the histologic type. The data were compared using both one-way analysis of variance (ANOVA) (assessing the three histologic types separately) and the independent t-test (dichotomizing histologic subtypes as epithelioid versus non-epithelioid). Histologic type was present as follows: the epithelioid cell was n = 4, and the spindle cell was n = 11, the mixed cell type was n = 11. The mean ADC was 1.06 ± 0.24 × 10-3 mm2/s in the epithelioid cells, 0.98 ± 0.19 × 10-3 mm2/s in the spindle cells and 0.96 ± 0.26 × 10-3 mm2/s in the mixed cell type. No significant difference in the mean ADC value of the histopathologic subtypes was found, either when assessing the three histologic types separately (p = 0.76) or after dichotomizing the histologic subtypes as epithelioid and non-epithelioid (p = 0.82). DWI-ADC is not accurate enough to distinguish histologic types of UMs.

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PURPOSE: To compare the diagnostic value of ultrasonic elastosonography (UE) and contrast-enhanced ultrasonography (CEUS) for benign and malignant intraocular tumors. METHODS: This retrospective study enrolled patients with intraocular tumors at Beijing Tongren Hospital, Capital Medical University (August 2016 to January 2020). The strain rate ratio (strain rate of tumor tissue divided by strain rate of surrounding normal tissue) was measured by UE. CEUS was performed using SonoVue® contrast agent. The performance of each method at differentiating benign from malignant intraocular tumors was evaluated by receiver operating characteristic curve analysis. RESULTS: The analysis included 147 eyes in 145 patients (45.6 ± 13.4 years-old; 66 males): 117 patients (119 eyes) with malignant tumors and 28 patients (28 eyes) with benign tumors. At an optimal cutoff of 22.67 for the strain rate ratio, UE distinguished benign from malignant tumors with a sensitivity of 86.6% and a specificity of 96.4%. CEUS showed that 117 eyes with malignant tumors had a fast-in, fast-out time-intensity curve, and only two eyes with malignant tumors had a fast-in, slow-out curve, while all 28 eyes with benign tumors had a fast-in, slow-out curve. CEUS differentiated benign from malignant tumors with a sensitivity of 98.3% and a specificity of 100%. The diagnostic results differed significantly between the two methods (P = 0.004, McNemar test). The diagnostic performances of the two tests were moderately consistent (κ = 0.657, P < 0.001). CONCLUSION: Both CEUS and UE have good diagnostic value in the differentiation of benign intraocular tumors from malignant intraocular tumors.

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ABSTRACT The aim of this study was to alert the ophthalmic community to an atypical manifestation of ocular surface squamous neoplasia, which may delay diagnosis and treatment and result in a guarded visual prognosis and significant sequelae. A 61-year-old immunocompetent man presented with an initial diagnosis of necrotizing scleritis in the right eye for 3 months. He was treated with systemic prednisone but experienced persistent pain and low visual acuity. Conjunctival biopsy of the affected region confirmed the diagnosis of invasive ocular surface squamous neoplasia, which progressed with intraocular and orbital invasion; thus, exenteration was performed. Masquerade syndrome should be suspected in patients with nodulo-ulcerative lesions of the conjunctiva and sclera. This clinical can be more aggressive, with a greater likelihood of intraocular and orbital involvement. The earlier the diagnosis and treatment, the better the patient prognosis.

RESUMO O objetivo é alertar a comunidade oftalmológica sobre uma manifestação atípica de neoplasia escamosa da superfície ocular (OSSN) que pode levar a um atraso no diagnóstico e tratamento, evoluindo com prognóstico reservado e significativas sequelas. Homem, imunocompetente, 61 anos com diagnóstico inicial de esclerite necrosante em olho direito há 3 meses, em tratamento com prednisona sistêmica porém com persistência da dor e baixa acuidade visual. Realizado biópsia conjuntival em região acometida e diagnosticado como neoplasia escamosa da superfície ocular invasiva. Evolui com invasão intraocular e orbital sendo submetido a exenteração. Assim sendo, deve-se suspeitar de síndrome mascarada frente a um paciente com lesões nódulo-ulcerativas da conjuntiva e esclera. Essa forma clínica pode ser mais agressiva, com maior chance de comprometimento intraocular e orbital. Quanto mais precoces o diagnóstico e o tratamento, melhor o prognóstico para o paciente.

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ABSTRACT Purpose: To evaluate the impact of corneal and conjunctival tumors on the ocular surface and quality of life of patients before and after surgical treatment. Methods: This prospective study conducted a preoperative and 30- and 90-day postoperative assessment of patients diagnosed with conjunctival and corneal tumors. Demographic data were collected preoperatively. The 12-Item Short-Form Health Survey (SF-12) and Ocular Surface Disease Index (OSDI) questionnaires were applied to assess patients' quality of life and perception of their vision-related functions. The tear breakup time and Schirmer tests were performed for ocular surface evaluation. The tumor extensions were measured using ImageJ image analysis software. Results: Twenty-three patients were enrolled. The mean age at examination was 52.8 ± 17.3 years (range: 27-9 years). The most common tumor type was squamous cell carcinoma (61.5%). The patients' visual acuity improved significantly at 1 month and 3 months (p=0.018 and p=0.036, respectively). No significant differences were found between tear breakup time and Schirmer tests preoperatively and at 3 months postoperatively (p=0.150 and p=0.490, respectively). The SF-12 scores demonstrated significant differences between the preoperative and 30- and 90-day postoperative mental components (p=0.008 and p=0.026, respectively). Tumor extension was 868.7 ± 344.9 pixels (range, 224.6-1481.6 pixels) and were significantly correlated with the preoperative (p=0.011), 30-day postoperative (p=0.017), and 90-day postoperative (p=0.012) SF-12 mental components, as well as the emotional component at the 30th postoperative day (p=0.016). Conclusion: Patients with corneal and conjunctival tumors improved their ocular symptoms, visual acuity, and the emotional component of their quality of life after surgical excision of the tumor.

RESUMO Objetivo: Avaliar o impacto dos tumores cór­neo-conjuntivais na superfície ocular e na qualidade de vida dos pacientes antes e após o tratamento cirúrgico. Métodos: Este estudo prospectivo conduziu uma avaliação pré-operatória e com 30 e 90 dias de pós-operatório de pacientes com diagnóstico de tumores de córnea e conjuntiva. Os dados demográficos foram coletados no pré-operatório. Os questionários Health Survey Short-Form (SF-12) e Ocular Surface Disease Index (OSDI) foram aplicados para avaliar a qualidade de vida dos pacientes e a percepção de suas funções relacionadas à visão. Os testes tear break-up time (TBUT) e Schirmer foram realizados para avaliação da superfície ocular. A extensão do tumor foi medida usando o programa ImageJ. Resultados: Vinte e três pacientes foram incluídos. A média de idade foi de 52,8 ± 17,3 anos (27-79 anos). O tipo mais comum de tumor foi o carcinoma de células escamosas (61,5%). A acuidade visual dos pacientes melhorou significativamente em 1 mês e 3 meses (p=0,018 e p=0,036, respectivamente). Não houve diferenças significativas entre os testes tear break-up time e Schirmer no pré-operatório e com 3 meses de pós-operatório (p=0,150 e p=0,490, respectivamente). Os escores do SF-12 demonstraram que o componente mental apresentou diferença estatisticamente significante entre o pré-operatório e no 30 e 90 dias de pós-operatório (p=0,008 e p=0,026, respectivamente). A extensão do tumor foi de 868,7 ± 344,9 pixels (intervalo, 224,6-1481,6 pixels) e foram significativamente correlacionados com o componente mental de SF-12 no pré-operatório (p=0,011), 30 (p=0,017) e 90 dias de pós-operatório (p=0,012), e o componente emocional no 30º dia de pós-operatório (p=0,016). Conclusão: Pacientes com tumores córneo-conjuntivais melhoraram os sintomas oculares, a acuidade visual e o componente emocional da qualidade de vida após a excisão cirúrgica do tumor.

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RESUMO A metástase ocular é uma patologia incomum, principalmente quando se considera a íris como sítio da metástase. Apesar de incomum, a metástase ocular possui o câncer de mama como um de seus principais sítios primários. Ocasionalmente, a manifestação oftalmológica pode ser primária a qualquer outra clínica. Este artigo relata o caso de uma paciente com carcinoma de mama ductal invasivo com presumida metástase em corpo ciliar e íris em olho direito. O diagnóstico precoce interfere de forma significativa na evolução do quadro, sendo necessário o acompanhamento oftalmológico.

ABSTRACT Ocular metastasis is an uncommon pathology, especially when considering the iris as the site of metastasis. Although uncommon, eye metastasis has breast cancer as one of its main primary sites. Occasionally, the ophthalmologic manifestation may be primary to any other clinical manifestation. This article reports the case of a patient with invasive ductal breast carcinoma with presumed metastasis in the ciliary body and iris in the right eye. Early diagnosis significantly affects the evolution of the condition, requiring ophthalmologic follow-up.

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BACKGROUND: Choroid, ciliary body, and iris melanomas are often grouped as uveal melanoma, the most common intraocular primary malignancy. The purpose of the current study was to analyze the tumor profile of newly diagnosed cases of choroidal melanoma at a reference center in Sao Paulo, Brazil, and to investigate the frequency of eyes treated by enucleation that could have been treated with brachytherapy if available in the service. METHODS: Medical records of patients referred to our service with initial diagnostic hypothesis of choroidal melanoma from July 2014 to June 2020 were analysed on demographics, diagnosis confirmation, tumor measurement by ultrasonography and established treatment. Data were evaluated on clinical and demographic characteristics as age, sex, affected eye, ultrasound parameters, and treatment management of patients with clinically diagnosed choroidal melanoma. Among the patients submitted to enucleation, we investigated how many could have been selected to receive brachytherapy. RESULTS: From the 102 patients referred with the choroidal melanoma diagnosis hypothesis, 70 (68.62%) were confirmed. Mean measurements from the tumors in millimetres were: 9.19 ± 3.69 at height and 12.97 ± 3.09 by 13.30 ± 3.30 at basal. A total of 48 cases (68.57%) were enucleated, 8 (11.43%) were treated by brachytherapy in a different service, and 14 patients (20.00%) returned for enucleation at their original referral center. Out of the 48 patients enucleated, 26 (54.17%) could have been selected to brachytherapy treatment. CONCLUSIONS: The results indicate a late diagnosis of choroidal melanoma cases referred to our service. Most enucleated cases could have been treated with brachytherapy if it was broadly available at the national public health insurance. Further public health political efforts should focus on early diagnosis and better quality of life post-treatment for oncologic patients.

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BACKGROUND: Retinoblastoma is the most common malignant eye tumor in children and is associated with tumor predisposition syndrome (RB1 mutation) in up to 40% of cases. Imaging is an important part of the diagnostic workup of children with retinoblastoma both during the initial diagnosis and follow-up. OBJECTIVES: The goal of this review is to present the current state-of-the-art regarding imaging of children with retinoblastoma, including technical background and diagnostic clues with a brief discussion of future prospects. In addition, we summarize the general clinical diagnostic workup and therapeutic options. MATERIALS AND METHODS: Review of the literature and our own experience in the imaging of retinoblastoma. CONCLUSION: High-resolution magnetic resonance imaging (MRI) is the imaging modality of choice in children with retinoblastoma for diagnosis (estimation of diagnosis/differential diagnosis, evaluation of local and intracranial tumor extension) and during follow-up. Despite the characteristic calcifications, computed tomography (CT) examinations are no longer indicated in these patients. Due to the high association with tumor predisposition syndrome, genetic counselling is recommended.

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Introduction: Retinoblastoma greatly impacts the affected children and adolescents and their families. Epidemiological approaches to this form of cancer in Brazil are scarce, and this gap motivated this study. Objectives:This study aimed to describe the sociodemographic, clinical, epidemiological, and care characteristics of children and adolescents treated for retinoblastoma in Brazil and analyze whether there is any association between them. Methods: It is a cross-sectional, descriptive-analytical study, carried out with accompanying family members of children and adolescents treated for retinoblastoma in Brazil, sampled by snowball sampling, via a web survey, based on a semi-structured questionnaire elaborated by the authors, shared on social media. Results: The children and adolescents were represented by 129 accompanying family members from the five largest regions of Brazil. Low education level, housewifery, and low family income were associated with exclusive care provided by the Unified Health System, while low family income was associated with the use of ocular prosthesis post retinoblastoma. Conclusion: The identified odds ratio indicated the importance of family members' sociodemographic aspects for the clinical, epidemiological and care outcomes of the children and adolescents treated. Public healthcare services were the most used for oncology care, which endorses the relevance of the Unified Health System for children, adolescents, and their family members who have been impacted by retinoblastoma in the country (AU)

Introdução: O retinoblastoma impacta sobremaneira as crianças, adolescentes e familiares acometidos. Abordagens epidemiológicas sobre tal câncer no Brasil são escassas, lacuna esta que motivou este trabalho. Objetivos: Os objetivos desse estudo foram descrever as características sociodemográficas, clínicas, epidemiológicas e assistenciais de crianças e adolescentes atendidos por retinoblastoma no Brasil e analisar se existe associação entre tais características. Métodos: Tratou-se de um estudo transversal, descritivo-analítico, realizado com acompanhantes familiares de crianças e adolescentes atendidos por retinoblastoma no Brasil, amostrados por snowball sampling, via web survey, a partir de um roteiro autoral semiestruturado, compartilhado em mídias sociais. Os dados foram analisados por meio de estatística descritiva e inferencial, com o auxílio do software Statistical Package for the Social Sciences. Resultados: As crianças e os adolescentes foram retratados por 129 acompanhantes familiares das cinco grandes regiões brasileiras. A escolaridade baixa, a ocupação do lar e a baixa renda familiar dos respondentes associaram-se ao atendimento exclusivo pelo Sistema Único de Saúde, sendo que a baixa renda familiar também associou-se ao uso de prótese ocular pós retinoblastoma. Conclusões: As razões de chances identificadas sinalizaram para a importância dos aspectos sociodemográficos dos familiares para os desfechos clínicos-epidemiológicos-assistenciais das crianças e dos adolescentes atendidos. Os serviços públicos foram os mais utilizados para a assistência oncológica, o que endossa a relevância do Sistema Único de Saúde para as crianças, adolescentes e familiares impactados pelo retinoblastoma no país (AU)

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Background and Objectives: Secondary ocular localizations of hematological malignancies are blinding conditions with a poor prognosis, and often result in a delay in the diagnosis. Materials and Methods: We describe a series of rare cases of ocular involvement in six patients with hematological malignancies, reportedly in remission, who presented secondary ocular localizations, challenging to diagnose. Two patients had an acute lymphoblastic leukemia (ALL) and developed either a posterior scleritis or a pseudo-panuveitis with ciliary process infiltration. One patient had iris plasmacytoma and developed an anterior uveitis as a secondary presentation. Two patients had a current systemic diffuse large B-cell lymphoma (DLBCL) and were referred either for intermediate uveitis or for papilledema and vitritis with secondary retinitis. Finally, one patient with an acute myeloid leukemia (AML) presented a conjunctival localization of a myeloid sarcoma. We herein summarize the current knowledge of ophthalmologic manifestations of extramedullary hematopathies. Results: Inflammatory signs were associated with symptomatic infiltrative lesions well displayed in either the iris, the retina, the choroid, or the cavernous sinus, from the admission of the patients in the ophthalmological department. These findings suggest that patients with ALL, AML, systemic DLBCL, and myeloma can present with ophthalmic involvement, even after having been reported as in remission following an effective systemic treatment and/or allograft. Conclusions: Early detection of hidden recurrence in the eyes may permit effective treatment. Furthermore, oncologists and ophthalmologists should be aware of those rare ocular malignant locations when monitoring patient's progression after initial treatment, and close ophthalmologic examinations should be recommended when detecting patient's ocular symptoms after treatment.

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Necrosis in uveal melanomas can be spontaneous or induced by radiotherapy. The purpose of our study was to compare the histopathologic and MRI findings of radiation-induced necrosis of a group of proton beam-irradiated uveal melanomas with those of spontaneous necrosis of a control group of patients undergoing primary enucleation. 11 uveal melanomas who had undergone proton beam radiotherapy, MRI and secondary enucleation, and a control group of 15 untreated uveal melanomas who had undergone MRI and primary enucleation were retrospectively identified. Within the irradiated and nonirradiated group, 7 and 6 eyes with histological evidence of necrosis respectively, were furtherly selected for the final analysis; the appearance of necrosis was assessed at histopathologic examination and MRI. Irradiated melanomas showed a higher degree of necrosis as compared with nonirradiated tumors. Irradiated and nonirradiated lesions differed based on the appearance and distribution of necrosis. Irradiated tumors showed large necrotic foci, sharply demarcated from the viable neoplastic tissue; nonirradiated tumors demonstrated small, distinct foci of necrosis. Radiation-induced necrosis, more pigmented than surrounding viable tumor, displayed high signal intensity on T1-weighted and low signal intensity on T2-weighted images. The hemorrhagic/coagulative necrosis, more prevalent in nonirradiated tumors (4 out of 6 vs. 1 out of 7 cases), appeared hyperintense on T2-weighted and hypointense on T1-weighted images. Our study boosts the capability to recognize radiation-induced alterations in uveal melanomas at MRI and may improve the accuracy of radiologists in the evaluation of follow-up MR examination after radiotherapy.

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ABSTRACT: Introduction: Retinoblastoma greatly impacts the affected children and adolescents and their families. Epidemiological approaches to this form of cancer in Brazil are scarce, and this gap motivated this study. Objectives:This study aimed to describe the sociodemographic, clinical, epidemiological, and care characteristics of children and adolescents treated for retinoblastoma in Brazil and analyze whether there is any association between them. Methods: It is a cross-sectional, descriptive-analytical study, carried out with accompanying family members of children and adolescents treated for retinoblastoma in Brazil, sampled by snowball sampling, via a web survey, based on a semi-structured questionnaire elaborated by the authors, shared on social media. Results: The children and adolescents were represented by 129 accompanying family members from the five largest regions of Brazil. Low education level, housewifery, and low family income were associated with exclusive care provided by the Unified Health System, while low family income was associated with the use of ocular prosthesis post retinoblastoma. Conclusion: The identified odds ratio indicated the importance of family members' sociodemographic aspects for the clinical, epidemiological and care outcomes of the children and adolescents treated. Public healthcare services were the most used for oncology care, which endorses the relevance of the Unified Health System for children, adolescents, and their family members who have been impacted by retinoblastoma in the country. (AU)

RESUMO: Introdução: O retinoblastoma impacta sobremaneira as crianças, adolescentes e familiares acometidos. Abordagens epidemiológicas sobre tal câncer no Brasil são escassas, lacuna esta que motivou este trabalho. Objetivos: Os objetivos desse estudo foram descrever as características sociodemográficas, clínicas, epidemiológicas e assistenciais de crianças e adolescentes atendidos por retinoblastoma no Brasil e analisar se existe associação entre tais características. Métodos: Tratou-se de um estudo transversal, descritivo-analítico, realizado com acompanhantes familiares de crianças e adolescentes atendidos por retinoblastoma no Brasil, amostrados por snowball sampling, via web survey, a partir de um roteiro autoral semiestruturado, compartilhado em mídias sociais. Os dados foram analisados por meio de estatística descritiva e inferencial, com o auxílio do software Statistical Package for the Social Sciences. Resultados: As crianças e os adolescentes foram retratados por 129 acompanhantes familiares das cinco grandes regiões brasileiras. A escolaridade baixa, a ocupação do lar e a baixa renda familiar dos respondentes associaram-se ao atendimento exclusivo pelo Sistema Único de Saúde, sendo que a baixa renda familiar também associou-se ao uso de prótese ocular pós retinoblastoma. Conclusões: As razões de chances identificadas sinalizaram para a importância dos aspectos sociodemográficos dos familiares para os desfechos clínicos-epidemiológicos-assistenciais das crianças e dos adolescentes atendidos. Os serviços públicos foram os mais utilizados para a assistência oncológica, o que endossa a relevância do Sistema Único de Saúde para as crianças, adolescentes e familiares impactados pelo retinoblastoma no país. (AU)

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Ocular choristoma is composed of ectopic tissues with normal structures. The pathogenesis still remains uncertain. Histopathologically, it is a dense connective tissue mixed with epidermal appendages, smooth muscle cells, mature adipose tissue, lacrimal glands, lymph nodes, skeletal muscle fibers, cartilage and bone. Because of its low incidence, most of published literature are case reports. The clinical manifestations are non-specific and we need to distinguish it from other ocular masses. The choice of surgical resection depends on the ocular symptoms, the effect on appearance, and the need for clinical confirmation. This paper reviews the epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, differential diagnosis and treatment of ocular choristoma.

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Ciliary body tumor is a rare intraocular tumor. Due to its unique anatomical location, its correct diagnosis and reasonable treatment are very difficult problems. In terms of diagnosis and differential diagnosis, ophthalmologists need to fully utilize the role of slit lamp microscope and transillumination experiment to capture secondary changes in the anterior segment caused by hidden ciliary body tumors, such as monocular localized cataract, lens indentation, and pigment dissemination, etc. Ophthalmological imaging methods, especially ultrasound biomicroscopy, can achieve the purpose of early detection and early diagnosis. According to the size, location and morphological characteristics of the tumor, a reasonable treatment plan is formulated. Since ciliary body tumors are mostly benign, the recurrence rate of local resection is low, which can satisfy the pathological diagnosis and preserve part of the patient's vision. Therefore, eye-preserving treatment should be advocated. However, enucleation remains the treatment of choice for tumors that are too large to be treated with local excision or radiation, eyes with refractory glaucoma, and tumors that do not respond to radiation therapy.

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Circular RNA (circRNA) is a new group of endogenous non-coding RNAs produced by back-splicing, which has multiple molecular functions such as acting as microRNA sponges, regulators of transcription and splicing, adaptors for protein-protein interaction. Recent studies have shown that circRNA play an essential role in development and progression of retinal microvascular dysfunction, diabetic retinopathy, age-related macular degeneration, proliferative vitreoretinopathy, eye diseases caused by hyperhomocysteine and ocular malignancy. In pathological conditions, the differential expression of circRNA alters the transcription and translation of corresponding genes, thus changing the activity and function of cells. CircRNA may become a new marker and prognostic indicator of fundus diseases, and its targeted intervention may also become a potential treatment for fundus diseases.

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Objective:To investigate the clinical characteristics, treatment and prognosis of primary vitreoretinal lymphoma (PVRL) diagnosed and treated in our hospital during the past 10 years.Methods:A retrospective clinical study. From 2011 to 2021, 126 eyes of 67 patients with PVRL who were diagnosed and treated in Department of Ophthalmology, Eye-ENT Hospital, Fudan University were included in the study. Among them, there were 23 males (34.3%, 23/67) and 44 females (65.7%, 44/67); the average age was 57.1 years. There were 59 cases with both eyes (88.1%, 59/67) and 8 cases with one eye (11.9%, 8/67). At the initial eye diagnosis, 22 cases had a clear history of primary central nervous system lymphoma (PCNSL); 5 cases were found to have intracranial lesions by head imaging examination; 40 cases had no central nervous system involvement. Twenty cases were treated with glucocorticoids due to misdiagnosed uveitis. All patients received intravitreal injection of methotrexate (IVM) treatment. The treatment regimen was twice a week in the induction period for 2 weeks, once a week in the consolidation period for 1 month, and once a month in the maintenance period. Patients with PCNSL or both eyes received concurrent systemic chemotherapy (chemotherapy), and some in combination with radiation therapy to the brain (radiotherapy). The mean follow-up time was 39.3 months. The clinical manifestations, treatment and prognosis of the patients were retrospectively analyzed. The visual acuity before and after treatment was compared by t test. Results:Among the 22 cases with a clear history of PCNSL at the initial eye diagnosis, the average time from intracranial diagnosis to eye diagnosis was 22.9 months. Among the 40 cases without central nervous system involvement at first, 14 cases (20.9%, 14/67) developed central nervous system lesions during follow-up period. The mean time from ocular diagnosis to intracranial diagnosis was 9.9 months. Among the 126 eyes, 42 eyes (33.3%, 42/126) had anterior segment inflammation. vitreous inflammation type, retinal type, and vitreous retinal type were 58 (46.0%, 58/126), 7 (5.6%, 7/126), and 61 (48.4%, 61/126) eyes, and 9 of them (7.1%, 9/126) had optic nerve involvement at the same time. Patients received an average of 12 IVM treatments. IVM combined with systemic chemotherapy in 59 cases (88.1%, 59/67), of which 16 cases were combined with brain radiotherapy. All patients achieved complete remission after completing the treatment cycle (100.0%, 67/67). After treatment, 21 eyes (16.7%, 21/126) had ocular recurrence; 22 (32.8%, 22/67) had intracranial recurrence; 8 cases (11.9%, 8/67) died. The mean progression-free survival of patients was 23.7 months; the mean survival time was 43.6 months; the 5-year overall survival rate was 72.5%.Conclusions:The manifestations of PVRL are complex and diverse, and most of them are accompanied by involvement of the central nervous system. It can be divided into vitreitis type, retinal type and vitreoretinal type, and the optic nerve can be involved at the same time; IVM combined with systemic treatment can completely relieve the disease.

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BACKGROUND: Little is known about the value of dynamic contrast-enhanced (DCE) in combination with diffusion-weighted imaging (DWI) for the differentiation of lacrimal gland tumors. PURPOSE: To evaluate the ability of DCE and DWI in differentiating lacrimal gland tumors. MATERIAL AND METHODS: DCE and DWI were performed in 72 patients with lacrimal gland tumors. Time-intensity curve (TIC) patterns were categorized as type A, type B, type C, and type D. Apparent diffusion coefficient (ADC) was measured on DWI. Then, the diagnostic effectiveness of TIC in conjunction with ADC was assessed using classification and regression tree (CART) analysis. RESULTS: Type A tumors were all epithelial; they could be further separated into pleomorphic adenoma sand carcinomas. Type B tumors were all non-epithelial tumors, which could be further separated into benign inflammatory infiltrates (BIIs) and lymphomas. Type C tumors contained both carcinomas and non-epithelial tumors, which could be diagnosed into carcinomas, BIIs and lymphomas. Type D tumors were all PAs. The mean ADC of epithelial tumors was significantly higher than that of non-epithelial tumors, and the mean ADC values were significantly different between PAs and carcinomas. Besides, the mean ADC value of BIIs was higher than that of lymphomas. Therefore, the CART decision tree made by ADC and TIC had a predictive accuracy of 86.1%, differentiating lacrimal gland tumors effectively. CONCLUSION: Combined DCE and DWI-MRI can efficiently differentiate lacrimal gland tumors which can be of help to ophthalmologists in the diagnosis and treatment of these tumors.

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ABSTRACT Objective: To assess the deaths caused by eye cancer from 2010 to 2019 in Brazil. Methods: Data were selected from SUS' Computer Department platform at the Ministry of Health, including death certificates, from 2010 to 2019, from all Brazilian states and the Federal District, filtering the codes C69.0 to C69.9 as the cause of death, according to the International Classification of Diseases, Tenth Revision. Results: There were 1,859 deaths from malignant neoplasm of eye and adnexa (C69), in Brazil, from 2010 to 2019, affecting 1,062 (57.1%) men. The site of neoplasm was unspecified (C69.9) in 719 cases, representing the most frequent etiology in the C69 group (38.67%). The malignant neoplasm of the orbit (C69.6) was the second most common cause of death (22.59%), followed by malignant neoplasm of retina (C69.2) (14.73%). Conclusion: The number of deaths due to malignant neoplasm of eye and adnexa slightly increased through the years of 2010 to 2019, in Brazil.

RESUMO Objetivo: Avaliar os óbitos causados por câncer ocular durante os anos de 2010 a 2019 no Brasil. Métodos: Os dados foram selecionados na plataforma do Departamento de Informática do SUS do Ministério da Saúde, incluindo declarações de óbito, durante os anos de 2010 a 2019, de todos os estados brasileiros e do Distrito Federal, filtrando os códigos C69.0 a C69.9 como causa básica de óbito, de acordo com a Classificação Internacional de Doenças e Problemas Relacionados à Saúde - 10ᵃ Revisão. Resultados: Houve 1.859 óbitos por neoplasia maligna de olho e anexos (C69), no Brasil, no período de 2010 a 2019, acometendo 1.062 (57,1%) homens. O sítio da neoplasia não foi especificado (C69.9) em 719 casos, representando a etiologia mais frequente no grupo C69 (38,67%). A neoplasia maligna da órbita (C69.6) foi a segunda causa mais comum de óbito (22,59%), seguida pela neoplasia maligna da retina (C69.2) (14,73%). Conclusão: O número de óbitos por neoplasia maligna de olho e anexos aumentou discretamente ao longo dos anos de 2010 a 2019, no Brasil.

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ABSTRACT Objective: To develop and examine the usability of a mobile application aimed at improving knowledge about ocular surface tumors among medical students, general practitioners and ophthalmologists. Methods: A cross-sectional quantitative study was carried out by a multidisciplinary team and developed in three stages: administration of a specific questionnaire to medical students, assessing the demand for the application, creation and development of the application in collaboration with the Technological Innovation Laboratory of Centro Universitário Christus and usability assessment. General practitioners and ophthalmologists were selected to answer the System Usability Scale questionnaire. Data were exported into the Statistical Package for the Social Sciences, version 20.0 Windows, for quantitative analysis. Results: The OncoEye application was developed for the iOS and the Android platforms and included four main menus: Glossary, Patients, Referrals and Queries. Most students (89.9%) considered the development of the application important for wider access to information about the topic. Most students (39.9%) were able to recognize ocular lesions and 26.1% could distinguish between benign and malignant conditions. System Usability Scale questionnaire responses revealed good usability, with an average score of 92.65. Users (100%) described the application as user-friendly and well- integrated. Conclusion: An innovative application for ocular surface tumors was created and developed. The application achieved good levels of acceptance and was described as user-friendly by users.

RESUMO Objetivo: Desenvolver e avaliar a usabilidade de um aplicativo para dispositivos móveis que aprimore o conhecimento de estudantes de medicina e de médicos sobre tumores da superfície ocular. Métodos: Foi realizado estudo transversal e quantitativo por uma equipe multiprofissional, consistindo em três etapas: realização de um questionário específico com estudantes de medicina, avaliando a necessidade de elaboração do aplicativo; criação e desenvolvimento do aplicativo pelo Laboratório de Inovação Tecnológica do Centro Universitário Christus e avaliação de usabilidade. Médicos generalistas e oftalmologistas foram selecionados para responder ao questionário System Usability Scale. Os dados foram exportados para o programa Statistical Package for the Social Sciences, versão 20.0, para Windows para análise quantitativa. Resultados: O aplicativo OncoEye foi desenvolvido com quatro menus principais: Glossário, Pacientes, Encaminhamento e Perguntas. Dentre os estudantes, 89,9% consideraram o desenvolvimento do aplicativo importante para orientação sobre o tema, 39,9% souberam reconhecer lesão ocular, e 26,1% puderam diferenciar lesão ocular maligna ou benigna. As respostas dos usuários à ferramenta System Usability Scale demonstraram boa usabilidade, com pontuação média de 92,65 (87,74 a 97,55). Todos os usuários consideraram o aplicativo de fácil manuseio e bem integrado. Conclusão: Um aplicativo inovador para tumores da superfície ocular foi criado e desenvolvido, apresentando boa aceitação e fácil manuseio pelos usuários.

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