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Uveal melanoma (UM) is the most common primary intraocular tumor in adults, with no standardized treatment for advanced disease. Based on preliminary bioinformatical analyses DTYMK and PARP1 were selected as potential therapeutic targets. High levels of both proteins were detected in uveal melanoma cells and correlated with increased tumor growth and poor prognosis. In vitro tests on MP41 (BAP1 positive) and MP46 (BAP1 negative) cancer cell lines using inhibitors pamiparib (PARP1) and Ymu1 (DTYMK) demonstrated significant cytotoxic effects. Combined treatment had synergistic effects in MP41 and additive in MP46 cell lines, reducing cell proliferation and inhibiting the mTOR signaling pathway. Furthermore, the applied inhibitors in combination decreased cell motility and migration speed, especially for BAP1-negative cell lines. Our hypothesis of the double hit into tumoral DNA metabolism as a possible therapeutic option in uveal melanoma was confirmed since combined targeting of DTYMK and PARP1 affected all tested cytophysiological parameters with the highest efficiency. Our in vitro findings provide insights into novel therapeutic avenues for managing uveal melanoma, warranting further exploration in preclinical and clinical settings. [ABSTRACT FROM AUTHOR]

Purpose: To report a novel case of clear cell sarcoma presenting as an intraocular mass in a young adult female. Observations: A 34-year-old female presented to the Retina Service as an emergency department referral for an intraocular lesion noted in the left eye. The patient endorsed progressively decreased vision in the left eye for three months duration. Fundus examination of the left eye was notable for a circumpapillary, retinal amelanotic mass with vitreous seeding. Due to concern for retinoblastoma and poor visual potential, the decision was made for primary enucleation. Initial histopathologic and immunohistochemical analyses demonstrated a primitive-appearing, epithelioid to spindled, intraretinal malignancy, with expression of all melanocytic markers by immunohistochemistry. Molecular genetic testing detected an EWSR1::ATF1 fusion. The patient was diagnosed with clear cell sarcoma of the eye and systemic work up revealed no additional disease burden. Conclusions and importance: Clear cell sarcoma is a soft tissue sarcoma that can present within the eye and should be included in the differential diagnosis of amelanotic intraocular (intraretinal) tumors, including those in adults with clinical appearances similar to retinoblastoma.

Abstract Background Ciliary body tumor is extremely rare and treatment is challenging. The aim of this study is to present our experience in treating this rare entity, especially large tumors with more than 5 clock hours of involvement, and to evaluate the surgical outcomes and complications of local resection via partial lamellar sclerouvectomy in four cases of ciliary body tumors in China. Methods Four patients with ciliary body tumors underwent partial lamellar sclerouvectomy between October 2019 and April 2023 in Shanghai General Hospital, China. Tumor features, histopathologic findings, complications, visual acuity, and surgical outcomes were reviewed at a mean follow-up of 20.8 months. Results Four patients with a mean age of 31.8 years were included in this study. The histopathological diagnosis was adenoma of non-pigmented ciliary epithelium (ANPCE), schwannoma, and multiple ciliary body pigment epithelial cysts. The mean largest tumor base diameter was 6.00 mm (range: 2.00–10.00) and the mean tumor thickness was 3.50 mm (range: 2.00–5.00). Preoperative complications included cataract in 3 (75%) eyes, lens dislocation in 2 (50%), and secondary glaucoma in 1 (25%). Temporary ocular hypotonia was observed in one case and no other postoperative complications were observed. At a mean follow-up of 20.8 months, the best corrected visual acuity increased in 3 eyes and was stable in 1 eye. Tumor recurrence was absent in all eyes. All patients were alive at the end of follow-up. Conclusions Local tumor resection via PLSU is useful in the treatment of ciliary body tumors, including large tumors occupying more than five clock hours of pars plicata. Surgery-related complications were manageable with adequate preoperative assessment and careful operation during surgery.

Background: Ciliary body tumor is extremely rare and treatment is challenging. The aim of this study is to present our experience in treating this rare entity, especially large tumors with more than 5 clock hours of involvement, and to evaluate the surgical outcomes and complications of local resection via partial lamellar sclerouvectomy in four cases of ciliary body tumors in China. Methods: Four patients with ciliary body tumors underwent partial lamellar sclerouvectomy between October 2019 and April 2023 in Shanghai General Hospital, China. Tumor features, histopathologic findings, complications, visual acuity, and surgical outcomes were reviewed at a mean follow-up of 20.8 months. Results: Four patients with a mean age of 31.8 years were included in this study. The histopathological diagnosis was adenoma of non-pigmented ciliary epithelium (ANPCE), schwannoma, and multiple ciliary body pigment epithelial cysts. The mean largest tumor base diameter was 6.00 mm (range: 2.00–10.00) and the mean tumor thickness was 3.50 mm (range: 2.00–5.00). Preoperative complications included cataract in 3 (75%) eyes, lens dislocation in 2 (50%), and secondary glaucoma in 1 (25%). Temporary ocular hypotonia was observed in one case and no other postoperative complications were observed. At a mean follow-up of 20.8 months, the best corrected visual acuity increased in 3 eyes and was stable in 1 eye. Tumor recurrence was absent in all eyes. All patients were alive at the end of follow-up. Conclusions: Local tumor resection via PLSU is useful in the treatment of ciliary body tumors, including large tumors occupying more than five clock hours of pars plicata. Surgery-related complications were manageable with adequate preoperative assessment and careful operation during surgery. Key messages: What is known 1. Ciliary body tumor is a rare intraocular tumor that is difficult to detect and determine the malignancy, sometimes leading to overdiagnosis and overtreatment such as eyeball enucleation. 2. Partial lamellar sclerouvectomy (PLSU) is used as a local resection method for iridociliary tumors but surgical procedure is challenging and limited by tumor size. What this paper adds 1. PLSU can also be used in the treatment of large iridociliary tumors occupying more than five clock hours of pars plicata. 2. Surgery-related complications were manageable with adequate preoperative assessment and careful operation during surgery. [ABSTRACT FROM AUTHOR]

Introduction: The treatment of iridociliary and choroidal melanoma relies on the patient’s systemic health, tumor size, location, related features, state of the opposing eye, and personal preferences. The two categories are radiation and surgical techniques. Transpupillary thermotherapy, plaque radiotherapy, charged particle irradiation, local resection, enucleation, orbital exenteration, and experimental nanoparticle therapy are all options for treating choroidal melanoma. Case Presentation: The method that entails creating a partial thickness circular, rectangular, or polyhedral scleral flap in the region covering the tumor after removing a portion of the extraocular muscles is the most popular method for local excision in choroidal or choroidal-ciliary body cancers. We discuss our experience treating iridociliary melanoma using block excision and stereotactic irradiation on a linear accelerator with TD 20.0 Gy. Conclusion: One of the treatment modalities is the combined treatment approach using stereotactic irradiation and tumor resection, and our results 1 year after therapy are comparable to the rates of local control and anatomic eye preservation to those achieved in studies of comparable uveal melanoma treatment modalities.

Introduction: The treatment of iridociliary and choroidal melanoma relies on the patient's systemic health, tumor size, location, related features, state of the opposing eye, and personal preferences. The two categories are radiation and surgical techniques. Transpupillary thermotherapy, plaque radiotherapy, charged particle irradiation, local resection, enucleation, orbital exenteration, and experimental nanoparticle therapy are all options for treating choroidal melanoma. Case Presentation: The method that entails creating a partial thickness circular, rectangular, or polyhedral scleral flap in the region covering the tumor after removing a portion of the extraocular muscles is the most popular method for local excision in choroidal or choroidal-ciliary body cancers. We discuss our experience treating iridociliary melanoma using block excision and stereotactic irradiation on a linear accelerator with TD 20.0 Gy. Conclusion: One of the treatment modalities is the combined treatment approach using stereotactic irradiation and tumor resection, and our results 1 year after therapy are comparable to the rates of local control and anatomic eye preservation to those achieved in studies of comparable uveal melanoma treatment modalities. [ABSTRACT FROM AUTHOR]

AIM: To evaluate the diagnostic value of panoramic immersion B-scan ultrasonography (Pano-immersion B-scan, PIB) in complex retinal detachment (RD), persistent hyperplastic primary vitreous (PHPV) and intraocular tumors. METHODS: The clinical data of 44 patients collected from May 2012 to December 2019 in Chinese PLA General Hospital was retrospectively studied. All of these patients underwent PIB of the eye, because it was difficult to diagnose by routine ocular fundus examination, conventional ultrasound or/and ultrasonic biomicroscope (UBM) due to opacity of refractive media, pupillary occlusion, large involvement or special location of the lesion. The imaging features of difficult cases in PIB were analyzed. The diagnosis accuracy rating of PIB were evaluated and contrasted with conventional ultrasound or UBM by the standard of intraoperative diagnosis or/and pathological results. RESULTS: According to intraoperative diagnosis or pathological results as gold standard, among the 44 cases, there were 19 cases missed diagnosis, misdiagnosed or difficult-to-diagnose by conventional ultrasound or UBM, including 4 cases of long-standing RD difficult to diagnose, 4 cases misdiagnosed, and 11 cases incompletely observed or miss diagnosed. The diagnostic accuracy rate of PIB and conventional ultrasound or UBM were 100% (44/44) and 56.82% (25/44), and the sensitivity of them were 100% and 56.82%. All the patients underwent PIB and were diagnosed as RD (15 cases), retinal and choroidal detachment (4 cases), subchoroidal hematocele (1 case), vitreous opacity and/or organic membrane formation (4 cases), PHPV (12 cases), iris and/or ciliary body tumors (3 cases), and choroidal tumors (6 cases). According to the intraoperative diagnosis or pathological results, the diagnostic coincidence rate of PIB was 100%, which was significantly higher than conventional ultrasound and UBM. CONCLUSION: PIB can help to accurately diagnose complex RD, PHPV, and intraocular masses with special location or/and excessive size. It has important diagnostic value for patients with equivocal findings at conventional ultrasound examination.

AIM: To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma. METHODS: The clinical and pathological data of 11 patients (11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital, Capital Medical University, from 2007 to 2021 were retrospectively analyzed. RESULTS: The initial symptoms of 11 patients included vision loss (6 eyes), atrophia bulbi (1 eye), proptosis (2 eyes), and leukocoria (2 eyes). Most patients suffered with corneal opacity, anterior chamber flare and hyphema. Iris neovascularization and synechia, complicated cataract, and secondary glaucoma occurred in several cases. Three patients even had lens subluxation and retinal detachment. B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball. Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces. All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence. In this study, 6 patients had enucleation (2 patients had systemic chemotherapy after surgery), and the other 5 patients had local tumor resection (1 patient had plaque radiotherapy after surgery). CONCLUSION: Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma. Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma, and can make an accurate diagnosis and better treatment.

Purpose: To examine the role of the strain ratio in elastosonography for the differential diagnosis of common intraocular tumors such as choroidal melanoma, choroidal hemangioma, choroidal metastatic carcinoma, and retinoblastoma. Methods: This study included patients suffering from intraocular space-occupying lesions and who visited Beijing Tongren Eye Center of Beijing Tongren Hospital affiliated to Capital Medical University from June 2016 to March 2020. All patients underwent a physical examination, fundus examination with mydriasis, color Doppler ultrasonography, elastosonography, magnetic resonance imaging (MRI), and fundus angiography within 1 week. All patients were grouped as choroidal melanoma, choroidal metastatic carcinoma, retinoblastoma, choroidal hemangioma, and optic disk melanocytoma. A receiver operating characteristic (ROC) curve analysis was performed to assess the strain ratio for diagnosing malignant intraocular tumors. Results: A total of 155 patients (161 eyes) were recruited. The strain ratios measured were 39.59 ± 15.92 for choroidal melanoma, 36.85 ± 13.64 for choroidal metastatic carcinoma, 38.93 ± 17.27 for retinoblastoma, 13.42 ± 10.93 for choroidal hemangioma, and 3.84 ± 1.32 for optic disk melanocytoma. The strain ratios of the three malignant lesions were significantly higher than those of the two benign lesions (all P < 0.001). The area under the ROC curve was 0.95 ± 0.028. The optimal cutoff point was 22.67, with 85.7% sensitivity and 96.4% specificity. Conclusion: There were significant differences in elasticity between the malignant and benign intraocular tumors. The strain ratio using elastosonography could serve as an important auxiliary examination to distinguish between benign and malignant intraocular tumors.

A 51-year-old Eastern Mediterranean male without known systemic medical illness referred to our clinic with a painless loss of vision for 2 months in the left eye. The diagnosis of choroidal malignant melanoma was suspected when fundus examination and ocular ultrasonography were done after the continuous deterioration in visual acuity. Orbital magnetic resonance imaging evaluation was consisted with choroidal melanoma and enucleation was performed. The post-operative histopathologic report confirmed the choroidal malignant melanoma. This report highlights the importance of B-scan ocular ultrasonography in evaluating an eye with deteriorating visual complaints. [ABSTRACT FROM AUTHOR]

Purpose: To examine the role of the strain ratio in elastosonography for the differential diagnosis of common intraocular tumors such as choroidal melanoma, choroidal hemangioma, choroidal metastatic carcinoma, and retinoblastoma. Methods: This study included patients suffering from intraocular space-occupying lesions and who visited Beijing Tongren Eye Center of Beijing Tongren Hospital affiliated to Capital Medical University from June 2016 to March 2020. All patients underwent a physical examination, fundus examination with mydriasis, color Doppler ultrasonography, elastosonography, magnetic resonance imaging (MRI), and fundus angiography within 1 week. All patients were grouped as choroidal melanoma, choroidal metastatic carcinoma, retinoblastoma, choroidal hemangioma, and optic disk melanocytoma. A receiver operating characteristic (ROC) curve analysis was performed to assess the strain ratio for diagnosing malignant intraocular tumors. Results: A total of 155 patients (161 eyes) were recruited. The strain ratios measured were 39.59 ± 15.92 for choroidal melanoma, 36.85 ± 13.64 for choroidal metastatic carcinoma, 38.93 ± 17.27 for retinoblastoma, 13.42 ± 10.93 for choroidal hemangioma, and 3.84 ± 1.32 for optic disk melanocytoma. The strain ratios of the three malignant lesions were significantly higher than those of the two benign lesions (all P < 0.001). The area under the ROC curve was 0.95 ± 0.028. The optimal cutoff point was 22.67, with 85.7% sensitivity and 96.4% specificity. Conclusion: There were significant differences in elasticity between the malignant and benign intraocular tumors. The strain ratio using elastosonography could serve as an important auxiliary examination to distinguish between benign and malignant intraocular tumors. [ABSTRACT FROM AUTHOR]

Retinoblastoma (Rb), the most frequent malignant intraocular tumor in childhood, is caused by mutations in the retinoblastoma gene (RB1) situated on chromosome 13q14.2. The incidence of retinoblastoma is approximately 1 in 17,000 live births with approximately 8,000 new cases diagnosed each year worldwide. Rb is the prototypical hereditary cancer in humans. Autosomal dominant inheritance is seen in 30-40% of cases whereas the non-inherited sporadic type accounts for the remaining 60-70%. Rb arises due to inactivation of both alleles of the Rb tumor suppressor gene, which results in a defective Rb protein (pRB) with subsequent cell cycle impairment and uncontrolled cell proliferation. Patients with Rb have survival rates higher than 95-98% in industrialized countries but mortality remains high in developing countries. For example, the mortality rate in Africa is 70%. In all cases of intraocular and extraocular retinoblastoma, there is a need for new therapies that are more effective and carry less risk of toxicity. The Bruckner test is a practical and easy test for the detection of Rb, this test consists of assessing the fundus reflex through the pupil (red reflex) in both eyes simultaneously with a bright coaxial light produced with the direct ophthalmoscope. Rb can be detected by the Bruckner test showing a pupil that shines white or “Leukocoria”. Although the diagnosis of Rb remains essentially clinical, the newly identified biomarkers could contribute to early molecular detection, timely detection of micrometastases and establish new therapeutic options for Rb. [ABSTRACT FROM AUTHOR]

Abstract Background To report a rare case of granular cell tumor invading the retina. Case presentation A 56-year-old female complained of blurred vision for 2 weeks in her left eye. An irregular-shaped retinal mass in the inferonasal and extending to the optic disc accompanied by dense exudation and extensive serous retinal detachment was observed. Several intravitreal bevacizumab injections were ineffective for stabilizing retinal exudation and intraocular pressure (IOP). Vitrectomy was performed to re-attach the retina and obtain a tumor biopsy specimen. Histopathological analysis revealed that the intraocular mass was a granular cell tumor. Immunohistochemical studies demonstrated that the tumor was positive for S100 and CD68, focal positive for neurofilaments, but negative for ERG and HMB-45. Local recurrence and distant metastasis were not found, but visual acuity had worsened to no light perception at the last visit due to uncontrolled intraocular pressure and retinal exudation after the surgery. Conclusions Granular cell tumor is a rare benign neoplasm, but it can lead to devastating visual loss if it invades the retina adjacent to the optic nerve head.

Retinoblastoma (Rb), the most frequent malignant intraocular tumor in childhood, is caused by mutations in the retinoblastoma gene (RB1) situated on chromosome 13q14.2. The incidence of retinoblastoma is approximately 1 in 17,000 live births with approximately 8,000 new cases diagnosed each year worldwide. Rb is the prototypical hereditary cancer in humans. Autosomal dominant inheritance is seen in 30-40% of cases whereas the non-inherited sporadic type accounts for the remaining 60-70%. Rb arises due to inactivation of both alleles of the Rb tumor suppressor gene, which results in a defective Rb protein (pRB) with subsequent cell cycle impairment and uncontrolled cell proliferation. Patients with Rb have survival rates higher than 95-98% in industrialized countries but mortality remains high in developing countries. For example, the mortality rate in Africa is 70%. In all cases of intraocular and extraocular retinoblastoma, there is a need for new therapies that are more effective and carry less risk of toxicity. The Bruckner test is a practical and easy test for the detection of Rb, this test consists of assessing the fundus reflex through the pupil (red reflex) in both eyes simultaneously with a bright coaxial light produced with the direct ophthalmoscope. Rb can be detected by the Bruckner test showing a pupil that shines white or “Leukocoria”. Although the diagnosis of Rb remains essentially clinical, the newly identified biomarkers could contribute to early molecular detection, timely detection of micrometastases and establish new therapeutic options for Rb.

Abstract Intravitreal methotrexate (MTX) has been proven to be an effective treatment for various intraocular diseases. In this article, a comprehensive review was performed on intravitreal applications of methotrexate. Different aspects of the administration of intravitreal MTX for various clinical conditions such as intraocular tumors, proliferative vitreoretinopathy, diabetic retinopathy, age-related macular degeneration, and uveitis were reviewed and the adverse effects of intravitreal injection of MTX were discussed. The most common indications are intraocular lymphoma and uveitis. Other applications remain challenging and more studies are needed to establish the role of intravitreal MTX in the management of ocular diseases.

Víctor Manuel Asensio-Sánchez, Gabriela Estefanía Pacheco-Carllirgos, Francisco Javier Valentín-Bravo Ophthalmology Department, Clinical University Hospital of Valladolid, Valladolid, SpainCorrespondence: Víctor Manuel Asensio-Sánchez Tel +34 983 37 79 82Email vmasensio@yahoo.esAbstract: This article describes a 63-year-old woman with a yellow-white mass in the inferior juxtapapillary region discovered on routine ocular examination. She was diagnosed as solitary idiopathic choroiditis (SIC) after an extensive ocular examination. SIC is a rare condition of unknown aetiology that can sometimes be mistaken as inflammatory processes or intraocular tumors. Solitary idiopathic choroiditis is suspected clinically and the diagnosis is established with multimodal imaging, so CIS has been renamed focal scleral nodule because of the scleral location.Keywords: solitary idiopathic choroiditis, unifocal helioid choroiditis, multimodal imaging, intraocular tumor, intraocular inflammation, focal scleral nodule

Purpose: Plaque therapy is a well-recognized treatment for intraocular tumors. In current study, we aimed to prospectively investigate the short-term effects of ruthenium 106 (Ru-106) plaque therapy on the cornea and ocular surface parameters. Methods: Twenty-five patients diagnosed with choroidal melanoma which undergone Ru-106 plaque therapy from 2016 to 2018 were included. Tear osmolarity, tear film break-up time, Schirmer test I, fluorescein dye staining based on Oxford staining method; Ocular Surface Disease Index (OSDI) questionnaire and corneal specular microscopy were performed. These tests were assessed preoperatively and then 3 months postoperatively. Results: The mean (±SD) age of subjects was 48.52 ± 15.18 years. The patients were followed for a mean(±SD) period of 3.64 ± 2.40 months. Total mean (+SD) delivered radiation dose to the tumor apex and total received radiation by the sclera was 83.20 ± 26.31 and 640.65 ± 472.69 Gray (Gy), respectively. In longitudinal analysis, OSDI score and Oxford staining score increased significantly (P = 0.002 for both variables) and the prevalence of dry eye disease (DED) increased from 20% preoperatively to 72% at 3 months postoperatively (P = 0.001).The changes in the all specular microscopy parameters were statistically nonsignificant (all P values > 0.05). Conclusion: There is a considerable increase in the rate of DED following plaque therapy for the treatment of choroidal melanoma in short-term follow-up. The OSDI questionnaire and fluorescein staining test are valuable tools for early detection of DED postoperatively.

Purpose: To present clinical images of a patient with neovascular glaucoma and hypermature cataract masking orbital extension of a uveal melanoma. Observations: A 67-year-old female was referred for neovascular glaucoma and found to have an intraocular tumor with massive orbital extension. She refused surgery and returned one year later with progression of the tumor with metastases and expired seven months later. Conclusions and importance: Unexplained glaucoma and cataract should be investigated for harboring underlying intraocular tumors to prevent delays in diagnosis. Providers also should obtain greater understanding of psychosocial and socioeconomic barriers to healthcare.

Massive retinal gliosis (MRG) is a rare condition of non-neoplastic glial proliferation, which forms massive lesions that fill the eye. MRG is commonly associated with phthisis bulbi (a non-functional eye), congenital anomalies, or malformations. Herein, we report a case of massive retinal gliosis associated with a traumatic phthisis bulbi, which was initially mistaken as a malignant intraocular tumor and confirmed only after an eye enucleation. A 70-year-old woman presented with a protruding ocular mass in her left eye which had slowly grown for a year. She had phthisis bulbi in her left eye due to trauma during her childhood. An orbital CT revealed an intraocular mass lesion with calcifications, raising the possibility of retinoblastoma or other malignant intraocular tumors. Enucleation of the left eye globe was performed. Histopathologic examination revealed exuberant proliferation of the glial cells, metaplastic bone formation, hyalinized vessels, and hyperplasia of the retinal pigment epithelium, confirming the diagnosis of MRG. Although rare, the possibility of MRG should be considered as a differential diagnosis when encountering an intraocular mass lesion, as it can be misdiagnosed as a malignant tumor. [ABSTRACT FROM AUTHOR]

Background: To report a rare case of granular cell tumor invading the retina.Case Presentation: A 56-year-old female complained of blurred vision for 2 weeks in her left eye. An irregular-shaped retinal mass in the inferonasal and extending to the optic disc accompanied by dense exudation and extensive serous retinal detachment was observed. Several intravitreal bevacizumab injections were ineffective for stabilizing retinal exudation and intraocular pressure (IOP). Vitrectomy was performed to re-attach the retina and obtain a tumor biopsy specimen. Histopathological analysis revealed that the intraocular mass was a granular cell tumor. Immunohistochemical studies demonstrated that the tumor was positive for S100 and CD68, focal positive for neurofilaments, but negative for ERG and HMB-45. Local recurrence and distant metastasis were not found, but visual acuity had worsened to no light perception at the last visit due to uncontrolled intraocular pressure and retinal exudation after the surgery.Conclusions: Granular cell tumor is a rare benign neoplasm, but it can lead to devastating visual loss if it invades the retina adjacent to the optic nerve head. [ABSTRACT FROM AUTHOR]

Purpose: Plaque therapy is a well-recognized treatment for intraocular tumors. In current study, we aimed to prospectively investigate the short-term effects of ruthenium 106 (Ru-106) plaque therapy on the cornea and ocular surface parameters.Methods: Twenty-five patients diagnosed with choroidal melanoma which undergone Ru-106 plaque therapy from 2016 to 2018 were included. Tear osmolarity, tear film break-up time, Schirmer test I, fluorescein dye staining based on Oxford staining method; Ocular Surface Disease Index (OSDI) questionnaire and corneal specular microscopy were performed. These tests were assessed preoperatively and then 3 months postoperatively.Results: The mean (±SD) age of subjects was 48.52 ± 15.18 years. The patients were followed for a mean(±SD) period of 3.64 ± 2.40 months. Total mean (+SD) delivered radiation dose to the tumor apex and total received radiation by the sclera was 83.20 ± 26.31 and 640.65 ± 472.69 Gray (Gy), respectively. In longitudinal analysis, OSDI score and Oxford staining score increased significantly (P = 0.002 for both variables) and the prevalence of dry eye disease (DED) increased from 20% preoperatively to 72% at 3 months postoperatively (P = 0.001).The changes in the all specular microscopy parameters were statistically nonsignificant (all P values > 0.05).Conclusion: There is a considerable increase in the rate of DED following plaque therapy for the treatment of choroidal melanoma in short-term follow-up. The OSDI questionnaire and fluorescein staining test are valuable tools for early detection of DED postoperatively. [ABSTRACT FROM AUTHOR]

A customized Collaborative Ocular Melanoma Study (COMS)-style eye plaque may provide superior dosimetric coverage compared with standard models for certain intraocular tumor locations and shapes. This work provides a recipe for developing and validating such customized plaques. The concept-into-clinical treatment process for a customized COMS-style eye plaque begins with a CAD model design that meets the specifications of the radiation oncologist and surgeon based on magnetic resonance, ultrasound, and clinical measurements, as well as a TG-43 hybrid heterogeneity-corrected dose prediction to model the dose distribution. Next, a 3D printed plastic prototype is created and reviewed. After design approval, a Modulay plaque is commercially fabricated. Quality assurance (QA) is subsequently performed to verify the physical measurements of the Modulay and Silastic and also includes dosimetric measurement of the calibration, depth dose, and dose profiles. Sterilization instructions are provided by the commercial fabricator. This customization procedure and QA methodology is demonstrated with a narrow-slotted plaque that was recently constructed for the treatment of a circumpapillary (e.g., surrounding the optic disk) ocular tumor. The production of a customized COMS-style eye plaque is a multistep process. Dosimetric modeling is recommended to ensure that the design will meet the patient's needs, and QA is essential to confirm that the plaque has the proper dimensions and dose distribution. The customized narrow-slotted plaque presented herein was successfully implemented in the clinic, and provided superior dose coverage of juxtapapillary and circumpapillary tumors compared with standard or notched COMS-style plaques. Plaque development required approximately 30 h of physicist time and a fabrication cost of $1500. Customized eye plaques may be used to treat intraocular tumors that cannot be adequately managed with standard models. The procedure by which a customized COMS-style plaque may be designed, fabricated, and validated was presented along with a clinical example. [ABSTRACT FROM AUTHOR]

Intravitreal methotrexate (MTX) has been proven to be an effective treatment for various intraocular diseases. In this article, a comprehensive review was performed on intravitreal applications of methotrexate. Different aspects of the administration of intravitreal MTX for various clinical conditions such as intraocular tumors, proliferative vitreoretinopathy, diabetic retinopathy, age-related macular degeneration, and uveitis were reviewed and the adverse effects of intravitreal injection of MTX were discussed. The most common indications are intraocular lymphoma and uveitis. Other applications remain challenging and more studies are needed to establish the role of intravitreal MTX in the management of ocular diseases. [ABSTRACT FROM AUTHOR]

The incidence of lung cancer with intraocular metastasis is low, of which choroidal metastasis is the most painful metastatic lesion. The clinical symptoms resulting from choroidal metastasis from lung cancer easily detected although they are rarely identified prior to the diagnosis of the primary malignancy. The quality of life of patients is inevitably impaired. Some lung cancer patients complain of ocular symptoms as the first manifestation of lung cancer. Early diagnosis and treatment can significantly overcome or delay the visual impairment and improve prognosis. The main therapeutic modalities include systemic and local treatments, while observation is also a treatment option. Currently, the feasibility and effectiveness of various treatment options are controversial worldwide. Herein, we summarize the underlying mechanisms, epidemiology, clinical features, auxiliary examinations, diagnosis, and recent treatment options for intraocular metastases. [ABSTRACT FROM AUTHOR]

To evaluate the distribution of the PAX8 transcription factor protein in ocular tissues and to investigate if immunohistochemical stains for this biomarker are useful in the diagnosis of intraocular tumors. Observational case series. Excision and cytologic analysis specimens of 6 ciliary body epithelial neoplasms, 2 iris epithelial neoplasms, 3 retinal pigment epithelial neoplasms, 3 intraocular medulloepitheliomas, 15 uveal melanomas, and 5 uveal melanocytomas. Hematoxylin–eosin and PAX8 immunohistochemical stains were performed on all specimens. In appropriate cases, bleached preparations and other immunohistochemical stains, including AE1/AE3 cytokeratin, Lin28A, and CD45, were performed. Distribution of PAX8 expression in normal and neoplastic tissue. Strong nuclear PAX8 expression was observed in the normal corneal epithelium, iris sphincter pupillae muscle, iris pigment epithelium and dilator muscle complex, nonpigmented and pigmented epithelia of the ciliary body, lens epithelium, and a subset of retinal neurons. The normal retinal pigment epithelium and uveal melanocytes did not stain for PAX8. The ciliary body epithelial and neuroepithelial tumors (adenoma, adenocarcinoma, and medulloepithelioma) showed uniform strong nuclear PAX8 immunoreactivity. All melanocytic tumors (iris melanoma, ciliary–choroidal melanoma, and melanocytoma) and retinal pigment epithelial neoplasms showed negative results for PAX8. A subset of tumor-associated lymphocytes, most prominent in uveal melanoma, showed positive results for PAX8. The uniformity of the PAX8 staining was superior to the variable cytokeratin staining in the ciliary epithelial neoplasms and the variable Lin28A staining in malignant medulloepithelioma. The veracity of PAX8 staining was equally as robust on cytologic analysis and open-flap biopsy specimens of ciliary epithelial and iris epithelial neoplasms, melanocytoma, and melanoma. PAX8 has proven to be a very useful diagnostic marker in a select group of adult intraocular tumors, and we highly recommend its inclusion in diagnostic antibody panels of morphologically challenging intraocular neoplasms. [ABSTRACT FROM AUTHOR]

Abstract Background Scleritis and/or uveitis sometimes accompanies patients who suffer from rheumatoid arthritis. However, few studies have reported scleritis and/or uveitis accompanying a fundus elevated lesion, such as an intraocular tumor. In this study, we report a case of rheumatoid uveitis associated with an intraocular elevated lesion. Case presentation A 66-year-old female visited another eye clinic and was diagnosed as bilateral anterior uveitis, and was prescribed steroid eye drops for treatment. She had previously been diagnosed as rheumatoid arthritis at the age of 30 years. Due to vitreous opacity that appeared in her right eye, we increased the instillation of steroid eye drops and the amount of oral prednisolone. Although the inflammation had improved, anterior uveitis relapsed, and an intraocular whitish elevated lesion resembling an intraocular tumor at the superior nasal retina appeared. We speculated this lesion to be a granuloma complicated with rheumatoid arthritis. Thus, we increased the amount of prednisolone administration, and the lesion began to shrink and ultimately fully disappeared. Conclusions We strongly believe that our case’s lesion was a subretinal granuloma related with rheumatoid arthritis, as it disappeared by increased corticosteroid treatment. Our findings show that we should consider rheumatoid arthritis in a differential diagnosis of such types of fundus elevated lesions.

This paper presents a review on retinal gliosis illustrated by series of three cases of patients (a 39-year-old man and a 35-year-old woman with massive retinal gliosis (MRG) and a 51-year-old man with truly focal nodular gliosis of retina) with intraocular tumor-like masses and loss of vision, who recently suffered from painful inflammation of eyeball and who classically had a history of remote ocular trauma, onset of blindness early in lifetime or gradual but progressive loss of sight. The diagnosis of this pathological entity is given for the lesions that are composed of GFAP strongly positive, elongated, fusiform cells consistent with fibrillary astrocytes. As illustrated in cases from our pathological practice, PAS gave positive patchy disseminated reaction in form of cellular densely purplish granules in minority of cells representing glycogen storing. This feature could be consistent with PAS-positive Müller cells that also constitute retinal gliosis as one of cellular components of normal retina that is induced to reactive proliferation. Thus, the paper presents histological background and differential diagnosis of the entity.

Abstract Background Granular cell tumor (GCT) can arise in any location in the body and present as a solitary, slow-growing, painless mass. However, GCT rarely affects the orbit. We report a Chinese girl who presented with an intraocular mass, and in whom a biopsy led to a diagnosis of GCT. Case presentation A 5-year-old Chinese girl exhibited exotropia in her right eye for 2 years and had been losing her vision for 6 months. The visual acuity in the right eye revealed no light perception. A fundus examination showed a large, yellowish-white, elevated, subretinal mass lesion in front of and inferior to the disc, with hemi-inferior-quadrant retinal detachment. The retina was greyish-yellow with scattered yellow spots. A vitrectomy with neoplasm resection was performed. A histopathologic examination revealed a GCT. The tumor cells were positive for CD68, NSE, S-100, and CD163 expression but negative for GFAP, Syn, and CD123 expression. The Ki-67 index was 1%. The right eye remained stable with visual acuity of no light perception at a 2-years follow-up. Conclusion Intraocular GCT can present as a yellow-white solid mass with no calcification. Although intraocular GCT is very rare, it can lead to devastating visual loss. Intraocular GCT should be kept in mind and considered in clinical practice.

Choroidal melanoma is a common intraocular malignancy among Caucasian adults. This serious and potentially lifethreatening lesion is associated with high rates of metastasis. This teaching case report describes an otherwise healthy young patient presenting with amelanotic choroidal melanoma. Differential diagnoses, symptoms, clinical findings, ancillary tests, treatment options and prognosis for amelanotic choroidal melanoma are discussed. Recognition of the clinical manifestations of choroidal melanoma leads to an early diagnosis and timely initiation of treatment. [ABSTRACT FROM AUTHOR]

Background: The typical imaged findings of retinoblastoma are an intraocular tumor with intratumoral calcification. Normal eye size is a supported finding of retinoblastoma. In practice, more than just a few cases had an altered eye size. Objective: To evaluate the effect of eye size in retinoblastoma. Materials and Methods: The present study included 47 patients with 54 diseased eyes. Twenty-seven patients underwent enucleation with histopathological results. The axial lengths (AL) and equatorial diameters (ED) were measured in both diseased and normal eyes. The imaging characteristics, tumor volume, and histopathological findings were recorded and analyzed. Results: The results showed no statistically significant differences between AL, ED, and calculated eye volumes (EV) between diseased and normal eyes. Anterior chamber depths were statistically shallower in retinoblastoma eyes (p<0.001). EV was weakly associated with tumor volumes. Large eye size was significantly related to choroidal invasion, massive choroidal invasion, scleral invasion, and optic nerve invasion in pathology (p=0.04, 0.03, 0.02, and 0.04, respectively). Conclusion: There were no statistically significant differences of eye size parameters in the eyes with retinoblastoma when compared to the normal eyes. Large eye size and large tumor volume are significantly related to invasive histopathological results. [ABSTRACT FROM AUTHOR]

Purpose: To discuss the clinical presentation and management of intraocular tumors masquerading as primary glaucoma or non-tumor-related secondary glaucoma. Methods: Retrospective chart review. Results: Ten patients with unsuspected intraocular tumor were referred to glaucoma clinic with a diagnosis of primary glaucoma or non-tumor-related secondary glaucoma. The mean age at referral was 25 years (median, 22 years; range, 1 day to 58 years). Referral diagnosis included neovascular glaucoma (n = 6), congenital glaucoma (n = 3), and angle-closure glaucoma (n = 1). The significant clinical signs included corneal edema (n = 3), megalocornea (n = 3), iris neovascularization (n = 4), hyphema (n = 2), and pseudohypopyon (n = 2). The mean interval between the onset of symptoms and the establishment of accurate diagnosis was 4 months (median, 3 months; range, 0.5–13 months). Two patients underwent inadvertent trabeculectomy, and one patient underwent evisceration prior to definitive diagnosis. The final diagnosis included uveal melanocytoma (n = 2), ciliary body medulloepithelioma (n = 2), choroidal melanoma (n = 2), retinoblastoma (n = 1), retinal capillary hemangioblastoma (n = 1), choroidal schwannoma (n = 1), and uveal metastasis (n = 1). The treatment modalities included plaque radiotherapy (n = 1), enucleation (n = 6), palliative systemic chemotherapy (n = 1), a combination of enucleation, systemic chemotherapy, and external beam radiotherapy (n = 1), and one patient was lost to follow-up. There was no evidence of death over a mean follow-up period of 13 months (median, 5 months; range, 2 weeks to 7 years). Conclusion: Unilateral raised intraocular pressure, iris neovascularization, or both may be the presenting features of intraocular tumors. High degree of suspicion and a thorough examination reveals the definitive diagnosis. [ABSTRACT FROM AUTHOR]

Objective: To report the surgical excision of an iridociliary adenoma and iridal melanocytoma using a postero-anterior cyclo-iridectomy in two dogs. Procedure: A 7 year old neutered male English springer spaniel (case 1) and a 7 year old neutered male Labrador mix (case 2) were presented for evaluation of an intrairidal mass OS. Results: Complete ophthalmic examination revealed a large, dorsonasal, well-demarcated, intrairidal mass OS. A tan to pink intrairidal mass extending into the iridocorneal angle (case 1) and a pigmented intrairidal mass (case 2) were present. B-mode ultrasonography showed a focal, soft tissue, homogenous mass within the uvea adjacent to and contacting the lens. Neither pars plana involvement nor vitreal extension was present. A postero-anterior cyclo-iridectomy was performed through a polyhedral scleral flap. Thermocautery was used to complete the cyclo-iridectomy (case 1) and partial iridectomy (case 2) to excise the mass en bloc. Histopathology revealed a completely excised iridociliary adenoma (case 1) and iris melanocytoma (case 2). The surgery sites healed without complication. Mild uveitis (cases 1 and 2), scant vitreal hemorrhage (case 1), and mild hyphema (case 2) were present three days postoperatively but had resolved ten days postoperatively. The patients remain visual twenty-two months (case 1) and seven months (case 2) postoperatively with a normal intraocular examination other than an iridal defect and mild dorsonasal lens capsular opacities. Conclusions: The surgical approach described in these cases is utilized in physicianbased medicine. This approach and the use of thermocautery provide a viable surgical option for excision of large iridociliary tumors in dogs. [ABSTRACT FROM AUTHOR]

Purpose: To present a rare presentation of optic nerve glioma (ONG) with total intraocular extension. Methods: A 44-year-old man with a history of loss of vision since childhood and recent development of progressive disfiguring proptosis was referred. Results: The vision in the affected eye was no light perception, and a proptosis of about 2–3 mm was present. Computed tomography and Magnetic Resonance Imaging (MRI) of brain/orbit showed a fusiform enlargement of the left optic nerve and total filling of vitreous cavity by a mass with high signal intensity in T2-weighted MRI. There was no extension into the intracranial cavity. The patient also had no signs of neurofibromatosis. Histopathology confirmed the diagnosis of ONG of the left orbit with extension into the globe and filling vitreous cavity. Conclusion: ONG may extend to the vitreous cavity with no simultaneous intracranial involvement.

Aim: To demonstrate a rare case of ciliary body leiomyoma in our patient Case report: A 72-year-old female reported to our clinic for a preventive examination, upon which we found a dome-shaped grey-brownish mass on the retinal periphery. After completing gonioscopic and ultrasound examinations, we referred the patient to a specialist facility. Due to a finding of suspicious malignant melanoma, we completed the MRI scan and recommended enucleation of the eyeball. A histopathological examination showed a leiomyoma of the ciliary body., Conclusion: The aim of this case report is to demonstrate the difficulty of intraocular leiomyoma diagnosis. Only immunohistochemical examination differentiated the tumor from malignant melanoma and determined the diagnosis of ciliary body leiomyoma. Perhaps because of the extreme rarity of this type of tumor, we often neglect to consider a diagnosis of leiomyoma.

The objective of this paper is to describe clinical behavior, histopathologic features, and immunohistochemical staining of two-related horses with intraocular teratoid medulloepithelioma. Two-related Quarter Horses with similar intraocular masses presented to the UF- CVM Comparative Ophthalmology Service for evaluation and treatment. The first horse, a 3-year-old gelding, had glaucoma and a cyst-like mass in the anterior chamber. Enucleation was performed. Histopathology revealed a teratoid medulloepithelioma. The tumor was considered to be completely excised. Fifteen months later, the gelding presented with swelling of the enucleated orbit and local lymph nodes with deformation of the skull. Cytology revealed neuroectodermal neoplastic cells. Necropsy confirmed tumor metastasis. Six weeks later, a 9-year-old mare, a full sibling to the gelding, presented for examination. An infiltrative mass of the iris and ciliary body was found that extended into the anterior, posterior, and vitreal chambers. Uveitis was present, but secondary glaucoma was not noted. Enucleation was performed and the histopathologic diagnosis was also teratoid medulloepithelioma. The mare has had no recurrence to date, 2 years following enucleation. Metastasis of intraocular teratoid medulloepithelioma is possible. Staging is recommended in cases where the diagnosis of teratoid medulloepithelioma is confirmed. Surveillance of full siblings is recommended until more information regarding etiology is known. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the anti-proliferative effect of puerarin on retinoblastoma cells. Methods: The effect of puerarin was examined on human retinoblastoma Y79 cells using cell proliferation assays and reverse transcription-polymerase chain reaction (RT-PCR). The effect of puerarin on the cell cycle was also investigated. Western blot and RT-PCR analyses were also performed to identify the putative mechanism of action. Results: The results showed that cell viability was suppressed by puerarin in a concentrationdependent manner with a half-maximal inhibitory concentration (IC50) of 0.184 ± 0.034 µmol/L. Moreover, puerarin increased the proportion of cells in G1 phase from 42.6 ± 3.1 to 62.83 ± 4.1, 75.76 ± 3.4 and 91.33 ± 5.1 % in a concentration-dependent manner at concentrations of 0.1, 0.2, and 0.4 µmol/L, respectively. The results also indicate that Bmi-1 mRNA and protein levels decreased after puerarin treatment. Additionally, flow cytometry data showed that Bmi-1 knock-down through siRNA resulted in G1-cell cycle arrest. The proportion of cells in G1 were 51.2 ± 2.5 and 71.4 ± 4.5 % for control and Bmi-1 siRNA-treated groups, respectively. Conclusions: The results show that puerarin exert suppressive effects on human retinoblastoma Y79 cells and therefore may find application in the treatment of intraocular tumor. [ABSTRACT FROM AUTHOR]

Pur po se: To evaluate the patients with intraocular tumors who underwent diagnostic fine-needle aspiration biopsy. Ma te ri al and Met hod: Between May 2003 and June 2009, fine-needle aspiration biopsy was performed in 14 patients with an intraocular tumor. For 6 cases with iris, iridocorneal angle or iridociliary pathology, aspiration was done with a 26-gauge needle under the surgical microscope. For 6 of the 8 cases with vitreous, retinal, optic disc or choroid pathology, aspiration was done with 20 D lens, indirect ophthalmoscope and 22-gauge needle. For the remaining 2 cases, aspiration biopsy was done by using 20-gauge pars plana vitrectomy and 22-gauge needle. A histopathologist examined the aspiration material in the operating room during the procedure. Re sults: Fine-needle aspiration biopsy of 14 patients showed that 3 had optic nerve melanocytoma, 2 had chorodial melanoma, 1 had iridociliary medulloepithelioma, 1 had iris melanoma, 1 had trabecular meshwork melanoma, 1 had iris nevus, 1 had leukemic infiltration, 1 had lymphoma, 1 had neuroendocrine carcinoma,1 had xanthogranuloma, and 1 patient had normal fine-needle aspiration biopsy findings. Dis cus si on: Fine-needle aspiration biopsy and cytopathology have an important role in the diagnosis of intraocular tumors that can not be reliably diagnosed by clinical examination. (Turk J Ophthalmol 2012; 42: 408-11)

We report the case of a 50-year-old woman with a history of diabetes mellitus who underwent left breast lumpectomy and ipsilateral lymphadenectomy in 1994 because of an infiltrating ductal carcinoma. Chemotherapy followed by radiotherapy to the breast and nodal areas were performed. In 2010, in a routine screening for diabetic retinopathy, two choroidal elevated masses above and below the optic nerve associated to serous retinal detachment of her right eye were noted. The patient was asymptomatic. Carcinoma was positive for hormone receptor. Hormone treatment with letrozole was established. Complete regression of the choroidal metastasis was observed 3 months later. Ophthalmologic screening in asymptomatic patients with breast cancer has the advantage of being a noninvasive procedure and enables an early treatment in isolated cases. However, some studies are an argument against the usefulness of eye screening due to the low incidence of asymptomatic choroidal metastasis and the cost that involves performing it routinely in a large number of patients. Aromatase inhibitors are well-tolerated drugs that may be a powerful tool in the management of metastatic breast cancer that express hormone receptors.

Objective: This study determined the clinical characteristics of retinoblastoma (RB) from 1998 to 2008 and compared the epidemiological and clinical patterns with those of the period from 1967 to 2001. Methods: We reviewed the clinical records of 152 patients with RB from 1998 to 2008 in terms of demographic and ophthalmological data and clinical staging or classification. Results: Sixty-three percent of cases were unilateral and 37% were bilateral. Three (3%) of 95 unilateral cases and 7 (12%) of 57 bilateral cases had family history of RB (p = 0.038). The mean age at onset was 17.8 months for unilateral and 7.4 months for bilateral cases, while the mean age at diagnosis was 26.4 months and 13.7 months respectively. The delay from onset to diagnosis was 69% in unilateral and 56% in bilateral RB groups. Financial cost (71.4%) was the leading reason for delay, followed by misdiagnosis (24.5%), and inaccessibility of medical facility (2.0%). The most common manifestations were leukocoria (77%), extraocular findings of orbital mass (9%), and proptosis (6%). Advanced intraocular stage was seen in 63 – 71.6% among those with unilateral and 56 – 60% in those with bilateral tumor. Conclusions: The onset of disease had not changed over the years, but patients in general were brought earlier for consultation. Most cases presented in the advanced stage. Decreasing the occurrence of extraocular RB through early consultation and treatment can improve patient survival.

Objective: The objective of this study was to determine the use of 3D printed model of an eye with intraocular tumor for linear accelerator-based stereotactic radiosurgery. Methods: The software for segmentation (3D Slicer) created virtual 3D model of eye globe with tumorous mass based on tissue density from computed tomography and magnetic resonance imaging data. A virtual model was then processed in the slicing software (Simplify3D®) and printed on 3D printer using fused deposition modeling technology. The material that was used for printing was polylactic acid. Results: In 2015, stereotactic planning scheme was optimized with the help of 3D printed model of the patient's eye with intraocular tumor. In the period 2001-2015, a group of 150 patients with uveal melanoma (139 choroidal melanoma and 11 ciliary body melanoma) were treated. The median tumor volume was 0.5 cm3 (0.2-1.6 cm3). The radiation dose was 35.0 Gy by 99% of dose volume histogram. Conclusion: The 3D printed model of eye with tumor was helpful in planning the process to achieve the optimal scheme for irradiation which requires high accuracy of defining the targeted tumor mass and critical structures. [ABSTRACT FROM AUTHOR]

RETINOBLASTOMA is the most common malignant intraocular tumor in children; it is also one of the most highly curable pediatric solid tumors if detected early. The conventional treatment of retinoblastoma is primary enucleation. Recent research reported a trend toward decreasing frequency of enucleation in the management of retinoblastoma.1 The trend toward globesparing interventions has been largely attributed to earlier diagnosis and recent success with conservative globe-sparing treatment options. Currently, globesparing interventions include first-line chemotherapy or chemoreduction, subconjunctival chemotherapy, systemic chemotherapy for metastasis, transpupillary thermotherapy (TTT), chemothermotherapy (CTT), laser photocoagulation, cryotherapy, brachytherapy, and external beam radiotherapy (EBRT). Expanded clinical options currently available have markedly decreased the overall enucleation rate for retinoblastoma.2 CLINICAL SCENARIO: A 10-month old boy is brought to an ophthalmologist because of cat’s eye reflex in the left eye. The patient had undergone enucleation of his right eye for glaucomatous stage retinoblastoma 6 months earlier. Examination revealed the presence of a solitary retinal mass of about 12 mm in diameter, located nasal to the disc. There was no evidence of vitreous seeding. Realizing that this was the only eye of the patient, the ophthalmologist wants to do everything humanly possible to preserve it. He has heard about chemothermotherapy (CTT) but is not sure if this was the best alternative he can offer. CLINICAL QUESTION: Among patients with retinoblastoma, is chemoreduction combined with adjuvant treatment effective in preserving the globe and vision? SEARCH METHOD: An electronic search of the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Group Trials Register) on The Cochrane Library (Issue 1 2005) and MEDLINE on PubMed was performed. The literature search was limited to the English language with no date restrictions. The search terms used were retinoblastoma and chemothermo-therapy, thermochemotherapy, or chemoreduction. The search yielded 70 articles, 22 were chosen for evaluation and 8 were included in the review. Two reviewers independently assessed the articles for inclusion. Selection criteria: This review was designed to include clinical trials in which treatment of retinoblastoma with chemoreduction combined with adjuvant therapy was compared with another treatment or no treatment. CITATIONS: 1. Schiavetti A, Hajistilianou T, Clerico A, et al. Conservative therapy in intraocular retinoblastoma: response/ recurrence rate. J Pediatr Hematol Oncol 2005; 27: 3-6.

Purpose: The goal of this study is to describe the presence of secondary cataract in patients with retinoblastoma treated at the National Institute of Pediatrics of Mexico (INP) over the past 10 years., Methods: This was a single center observational, retrospective and descriptive study. We included all eyes diagnosed with retinoblastoma and cataract between June 2011 and June 2021., Results: In total, 833 records of patients diagnosed with Retinoblastoma at the National Institute of Pediatrics during the period between June 2011 and June 2021 were reviewed. Out of all of them, only 14 developed cataract (1.6%). The median age at retinoblastoma diagnosis was 10.5 months (Rank: 6-13 months), and the median age at cataract diagnosis was 51.5 months (Rank: 25-73 months). The majority (13, 92.9%) of the patients had bilateral involvement. 42% of the eyes were Stage D according to the international classification of retinoblastoma. Cryotherapy was applied in 57.1%, intravitreal chemotherapy in 85.7%, radiation therapy in 42.6%, and only 7.1% of cases were treated with intra-arterial chemotherapy., Conclusions: The presence of cataract in patients with retinoblastoma is a rare but important entity impacting the development of vision in children and detection of intraocular tumors. These probably occur late as a result of the multiple treatments to which the children have been subjected, without being able to determine in this study which is the risk factor most associated with the development of this pathology., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

A 40-year-old female patient referred for a possible intraocular tumor was found to have an endogenous bacterial endophthalmitis in her right eye. Fundus examination revealed an amelanotic dome shaped choroidal mass and an exudative retinal detachment. Enhanced Depth Imaging-Optical Coherence Tomography (EDI-OCT), fundus autofluorescence (FAF) and ultrasounds were suggestive of a possible choroidal melanoma. A multimodal imaging approach and a thorough anamnesis were instrumental in establishing the correct diagnosis. [ABSTRACT FROM AUTHOR]

Aims: To report the management of vision-threatening complications of vasoproliferative tumors of the retina. Methods: Clinical records of 31 eyes of 30 patients treated at our centers from 1998 through 2013 were reviewed. The main outcome measures included: type of treatment, tumor regression, tumor relapse and final visual acuity. Results: Seventeen patients (57%) underwent vitrectomy for epimacular membranes (n = 10), rhegmatogenous retinal detachment (n = 2), vitreous hemorrhage (n = 2), tractional retinal detachment (n = 1), serous retinal detachment (n = 1) and subhyaloid hemorrhage (n = 1). After the initial treatment, 10 additional surgeries were performed for vitreoretinal complications. Tumor activity was treated in all eyes either with photocoagulation or cryotherapy. Control of tumor activity was achieved in all cases, after treatment of recurrences. There were no statistically significant differences between initial and final visual acuity (p = 0.437). Recurrence showed a statistically significant association with the presence of proliferative vitreoretinopathy (p = 0.024), tumor thickness (p = 0.026), basal diameter (p = 0.031), and use of photocoagulation alone as initial treatment (p = 0.006, logistic regression). Conclusion: In our series, more than half of vasoproliferative tumors of the retina required surgery as the initial treatment. Recurrence was associated with tumor size, presence of proliferative vitreoretinopathy, and use of photocoagulation alone as the initial treatment. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Purpose: Adenoma of the non-pigmented ciliary epithelium (ANPCE) is extremely rare. The aim of this study is to present our experience in treating this rare entity and to determine the long-term surgical outcomes of local tumor resection (partial iridocyclectomy with lamellar sclerouvectomy) in five cases of ANPCE in China. Patients and methods: The medical data of four women and one man ranging in age from 28 to 46 years (median, 38 years) with ANPCE were reviewed retrospectively at the Zhongshan Ophthalmic Center of Sun Yat-sen University, China. All patients had received local tumor resection and the diagnosis was confirmed by histopathological examination. Results: All five cases experienced blurred vision. The right eye was affected in four cases and the left in one. Slit-lamp biomicroscopy revealed a nodular, non-pigmented, gray-white mass with a slightly irregular surface. Tumor invasion through the peripheral iris was observed in two patients, two patients had multiple iris or ciliary cysts, and one patient had a partially bloodstained cornea. All tumors transmitted light readily. Ultrasound biomicroscopy showed a middle- or hyper-echoic solid mass in the ciliary body. MRI examination showed the tumor hyperintense to vitreous on T1WI and hypointense to vitreous on T2WI. Local resection was performed in all cases. After a median of 5.0 years of follow-up, no tumor recurrence was present in any of the cases and all involved eyes were saved. The visual acuity improved seven lines in three cases, decreased one line in one case, and vision was lost in one case. Conclusions: ANPCE often presents in adults as gray-white in color, with an irregular and sometimes multilobulated surface. The tumor transmits light well. Local resection of the mass generally provides the patient with useful vision. Recurrence after surgical removal is unlikely. [ABSTRACT FROM AUTHOR]

A 66-year-old woman presented with a blind, painful, hypertensive, and proptotic left eye. Computed tomographic imaging revealed a well-circumscribed mass involving the left orbit and globe. Metastatic work-up failed to reveal extraorbital lesions and the tumor was removed in toto via an evisceration approach orbitotomy. Histopathology and immunohistochemistry were most consistent with mammary-type myofibroblastoma with fascicles of bland, uniform spindle cells that stained positive for desmin and CD34. We are not aware of previous reports of orbital or ocular myofibroblastoma. This neoplasm has not been shown to recur, undergo malignant transformation, or metastasize. Familiarity with its clinical, histopathologic, and immunohistochemical features may improve diagnostic accuracy and treatment decisions for patients presenting with similar findings. [ABSTRACT FROM AUTHOR]

The human eye can be compromised by a variated spectrum of neoplasms and reactive processes. Here we present a rare case of a primary intraocular inflammatory myofibroblastic tumor (IMT) dependent on the sclera and choroid in a 31-year-old female. The knowledge surrounding IMTs, previously included in the category of inflammatory pseudotumors, has undergone dynamic changes in the past two decades. Here we review the characteristics of these tumors in the human eye and in the surrounding structures, and we describe the recent advances that allow molecular characterization of the neoplastic nature of this entity. [ABSTRACT FROM AUTHOR]