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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.

A clinical and morphological description of a case of pilocytic astrocytoma (astrocytic hamartoma) of the retina and optic nerve in an 11-year old girl is presented. The clinical and instrumental characteristics of the tumor are determined by ultrasound and MRI tests. Detailed cytological and pathomorphological description of the tumor, which is rarely observed, is given. The tumor poses a threat to the eye and requires that the eyeball be removed.

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.

La metástasis coroidea es el tumor intraocular más común en adultos. Estudios de autopsias sugieren que aproximadamente el 10% de pacientes con cáncer diseminado tiene metástasis ocular, más frecuentemente a la coroides. Presentamos el caso de una mujer de 37 años de edad quien consultó por disminución de la agudeza visual a consecuencia de una metástasis coroidea.

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.

OBJECTIVE: Retinoblastoma is the most common primary intraocular tumor affecting children. We examine the role of parental occupational exposures and risk of retinoblastoma among offspring. METHODS: Our population-based case-control study linked data from four nationwide Danish registries and included all cases of retinoblastoma diagnosed in Danish children (

Purpose. Retrospective analysis of clinical, instrumental, cytological and pathological features of intraocular medulloepithelioma based on our own experience in the period from 2005 to 2018. Patients and Methods. For a specified period of time in the department of ocular oncology and radiology in Moscow Helmholtz Research Institute of Eye Diseases we examined 9 patients aged from 1 to 44 years (5 men and 4 women), with a confirmed histological diagnosis of intraocular medulloepithelioma (medulloblastoma). The average age of patients in assessing vital status was 15.8 ± 13.5 years, the average age at the time of making the diagnosis “intraocular neoplasm” was 9.5 ± 11 years. All patients underwent a comprehensive clinical and instrumental ophthalmologic examination, which included visometry, tonometry, perimetry, biomicroscopy, ophthalmoscopy, Doppler ultrasound (ACUSON, USA), as well as general physical examination. Analysis of the initial status (before hospitalization) was carried out retrospectively according to the data of outpatient records. In one case, iridocyclosclerectomy was performed, in eight cases — enucleation of the affected eye followed by histopathological examination, as well as analysis of long-term results of treatment. Results . A detailed analysis of nine clinical cases is presented, including typical clinical features, echographic signs of a meduloepithelioma (heterogeneous structure of the tumor, anechoic inclusions (cysts) in the thickness of the tumor) are presented. According to the localization, we observed the tumor of the ciliary body ( n = 3), the ciliary body and iris ( n = 2), the retina ( n = 1), the retina, the choroid and the optic nerve disc ( n = 1), the ciliary body and the choroid ( n = 1) and the choroid with invasion in extraocular muscles and optic nerve ( n = 1). In 8 cases, enucleation was performed, one case was preceded by an iridectomy and a fine- needle aspiration biopsy. One patient underwent local conservative treatment (iridocyclosclerectomy) in the presence of a tumor of the ciliary body and iris. Pathological and cytological features of the tumor were presented. 8 of 9 patients were alive and had no signs of metastases. One patient died 3 years after treatment. Conclusions . Despite the relative rarity, medulloepithelioma should be included in the differential-diagnostic series in patients with suspected retinoblastoma, non-pigmented uveal melanoma, adenocarcinoma of the retinal pigment epithelium, and other rare intraocular neoplasms.

Uveal melanoma is the most common primary intraocular tumor with a poor prognosis. Currently, treatment for UVM is limited, and the development of drug resistance and tumor recurrence are common. Therefore, it is important to identify new prognostic biomarkers of UVM and explore their role in the tumor microenvironment. Pyroptosis is a way of cell programmed death, and related research is in full throttle. However, the role of pyroptosis in UVM is unclear. In this study, we constructed the prognosis model of pyroptosis-related genes of UVM. This model can accurately guide the prognosis of UVM, and different groups differ in immune infiltration. We further verified our results in cell experiments. To some extent, our study can provide new ideas for the diagnosis and treatment of UVM.

Background: Retinoblastoma is the most common primary intraocular tumor in children. Prune belly syndrome is a rare congenital disease affecting newborns. The combination of the two diseases has never been reported before. In this article, we present a case of a male infant diagnosed with prune belly syndrome (PBS) at birth. Case report: Patient underwent many surgeries to manage the resulting consequences of PBS. At the age of 7 months, he presented with left eye leukocoria. He was examined and diagnosed with retinoblastoma. The eye was enucleated, and the diagnosis was confirmed histopathologically. This is the first case to be described in the literature of such an association to the best of our knowledge. Key words: prune belly syndrome; retinoblastoma; Eagle-Barrett syndrome

Uveal Melanoma (UM) is the primary intraocular tumor most commonly found in adults. The combination of therapy, Brachytherapy, surgery, Tranpupillary Thermal Therapy (TTT), Proton Beam Theraphy has not produced satisfactory results. The discovery of HER2 receptors expressed by UM cells can be used as a specific antigen target for the treatment of CAR T-celss. However, the effectiveness of CAR T-cell immunotherapy in tumors results in immunosuppressive T cells caused by an increase in Programmed cell Death Ligand-1 (PD-L1). This literature review demonstrates the success of HER2-specific CART T Cells as UM therapeutic efforts capable of eliminating tumor cells. In addition, CRSPR / Cas9 PD-1 / PD-L1-blocking modified HER2-specific CAR T cells can be a gene innovation in UM sufferers. Further clinical trials are needed to prove the effectiveness of CRISPR / Cas9 PD-1 / PD-L1-Blocking modified HER2-specific CAR T Cells in the treatment of UM patients.Keywords: Uveal melanoma, CAR T cell, reseptor HER

Purpose: Choroidal osteoma, which typically affects young women, is a benign intraocular tumor composed of mature bone within the choroid. Tumor decalcification and subfoveal choroidal neovascularization often lead to poor visual acuity although the etiology is unknown. Choriocapillaris characteristics in choroidal osteoma also are unknown. Methods: We report 4 cases of choroidal osteoma with decalcification in which the choriocapillaris could be imaged by optical coherence tomography angiography (OCTA). Results: OCTA showed that the choriocapillaris structure was maintained in the calcified portion, whereas a loss occurred in parts of the decalcified portion in all cases. Conclusions: OCTA may be useful for understanding the pathological states of choroidal osteoma.

The optic disc pit is a congenital anomaly, presenting as a round or oval depression in the optic disc, 0.10.7 disc diameters in size, located mainly along its lower-temporal margin. Approximately in 45-75% of the eyes with congenital optic disc pit a serous detachment of neuroepithelium (NED) in the macular area develops, which could become a cause for an erroneous diagnosis of an intraocular tumor. The article presents a clinical case of the optic disc pit, which required a differential diagnosis with an intraocular tumor. It is shown that OCT signs of the optic disc pit are: the connection of optic disc structures with the subretinal space, as well as the presence of signs of invagination of the retinal nerve fiber layer in the optic disc structures.

Retinoma (or retinocytoma) is a rare benign intraocular tumor with characteristic features and can be diagnosed clinically. In this article, we present a case report and a review of literature about retinoma. The case is about an incidentally diagnosed retinoma in an asymptomatic young female who presented for vision checkup and was found to have an intraocular translucent-grey, elevated mass extending into the vitreous cavity from the retina with the characteristic features of retinoma. The main differential diagnosis included retinoblastoma and astrocytic hamartoma. The mass showed no growth over a period of 6 months of follow up with colored fundus photography and ultrasound. This case is, to the best of our knowledge, the first case of retinoma diagnosed in Jordan.

Retinoblastoma (RB) is the most common intraocular tumor of childhood. In low income countries, Time to diagnosis (TTD: interval between first symptom and diagnosis) has been associated with extrao...

Treatment abandonment is a leading cause of death in children with retinoblastoma worldwide. We studied children who abandoned treatment upfront at diagnosis to delineate the natural history of untreated retinoblastoma. Studied were children who received no treatment, diagnosed between 2007 and 2017 at 29 Chinese centers. Data were retrospectively collected from medical chart reviews and interviews with each patient’s family. During the study period, 44 children received no treatment after diagnosis of retinoblastoma. Clinical or radiologic evidence of orbital extension was available for 25 children, and radiologic evidence of systemic metastasis was available for 12 children. Median times from diagnosis of intraocular tumor to orbital disease was 13.7 months, orbital disease to metastasis was 2.6 months, and metastasis to death was 2.0 months. Children with brain metastasis had shorter survival than those with metastasis to other sites (median 1.0 vs. 3.1 months, p = 0.015). Overall, 36% of patients died within 12 months of diagnosis, 77% within 24 months, 95% within 36 months and 100% within 48 months. While multiple factors influence refusal of treatment, insights into the natural history of retinoblastoma derived from real-world evidence can inform clinicians and parents that retinoblastoma is life-threatening and encourage urgent treatment at diagnosis.

B-scan ultrasounds on 3 patients whose eyes were filled with cellular masses (two uveal melanomas and one reactive fibrous granulomatous proliferation) performed by 4 experienced retinal specialists were misinterpretated as containing no tumor mass. A possible explanation for this potentially avoidable oversight is offered.

PURPOSE 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. METHODS AND MATERIALS 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. RESULTS 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. CONCLUSION 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.

Approximately 5% of all melanoma tumors arise in ocular tissue. Most ocular melanoma (85%) is uveal (iris, ciliary body or choroid) in origin and it is the most common intraocular malignant tumor. Tumors arise from the pigment cells (melanocytes).

Retinoblastoma is an embryonal intraocular tumor that is nearly always diagnosed in the first 5 years of childhood. The overall survival of patients with retinoblastoma is high, but long-term sequelae are a major concern for many survivors. The most common late effects of retinoblastoma and its treatment are compromised vision or loss of the eye, ototoxicity after chemotherapy, cataract or cosmetic defects after radiotherapy, and psychological challenges.

Introduction Uveal melanoma (UM) is the most common primary intraocular tumor affecting adults. ~50% of patients will develop metastatic disease, mainly in the liver, for which there is currently no standard of care. The multi-faceted genetic etiology of UM informs prognosis and advises the clinical management of patients. Areas covered This review highlights the multifarious nature of the genetics that differentiate uveal nevi from UM, that initiate tumorigenesis and ultimately drive metastasis, and how these can be incorporated to multivariate models to predict whether and approximately when the metastatic disease will occur. Expert opinion Despite not being able to utilize current genomic biomarkers as therapeutically actionable targets in UM at present, advances in our understanding of this cancer, may lead to their inclusion to predict response to emerging therapies. Prognostication for individual patients is likely to progress from binary classifications of high- and low-metastatic risk, utilizing a multi-factorial approach to design molecular assays. Improvement in our comprehension of the genetic etiology of UM will bring us closer to the development of effective treatments.

Uveal melanoma (UM) is a type of intraocular tumor with a propensity to disseminate to the liver. Despite the identification of the early driver mutations during the development of the pathology, the process of UM metastasis is still not fully comprehended. A better understanding of the genetic, molecular, and environmental factors participating to its spread and metastatic outgrowth could provide additional approaches for UM treatment. In this review, we will discuss the advances made towards the understanding of the pathogenesis of metastatic UM, summarize the current and prospective treatments, and introduce some of the ongoing research in this field.

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.

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.

Uveal melanoma (UM), arising from melanocytes which reside in the uveal tract, is the most common primary intraocular tumor in adults. Despite successful treatment of the primary lesion by proton therapy or enucleation, up to 50% of UM patients develop metastases, predominantly in the liver. At present there are no effective systemic treatments for metastatic UM, 80% of patients will die within 1 year after diagnosis of the metastases. Therefore, better understanding of the UM disease is urgently needed for successful development of effective therapies.

Orbital cellulitis associated with retinoblastoma is uncommon and is characterized by noninfectious inflammation of the periorbital structures. The underlying mechanism is thought to be necrosis of the intraocular tumor, leading to intraocular and periorbital inflammation. We report 2 retinoblastoma patients who presented with an orbital cellulitis-like picture and discuss clinical characteristics, histopathologic features, and treatment.

Retinoblastoma represents the most common intraocular malignant tumor in children and worldwide is the most common intraocular malignancy. Uveal melanomas are the most common malignant intraocular tumors in adults. This review chapter includes key clinical features and important histopathologic findings for diagnosis and prognosis of both entities.

This section will discuss the role of enucleation and exenteration in the setting of the two most common primary intraocular tumors – retinoblastoma and uveal melanoma. The treatment of these tumors is complex and usually requires a multidisciplinary approach, which is beyond the scope of this chapter. Eye removal (enucleation or exenteration) is only one option of many for these patients and is the main focus of this chapter. Evisceration should never be performed if an eye has or is suspected of having an intraocular tumor.

Choroidal melanoma is the most common primary intraocular tumor but its incidence is only around 5 per million. In spite of the rarity of the tumor, well conducted clinical trials have significantly altered the management, improving the overall prognosis. This chapter highlights the key clinical trials that have brought a paradigm shift in the management of choroidal melanoma.

Retinoblastoma is the most common intraocular tumor in childhood. Topotecan has been widely used as an antineoplastic agent for retinoblastoma treatment. Topotecan penetration into living tumorspheres is quantified using confocal microscopy. This fluorescent drug dyes the living tissue and it can be recorded in a sequence of images over a period of time. The effective penetration of the drug depends on culture characteristics and requires a very specific timing. This penetration time is calculated empirically by an expert. The purpose of this work is to offer a statistical model to automatically estimate the penetration time of topotecan in the cell, based on the information obtained from a sequence of tumorsphere images.

Retinoblastoma is a common intraocular tumor of childhood. One of the medications used as an antineoplastic agent for retinoblastoma treatment is topotecan. Its penetration into living tumorspheres is quantified using confocal microscopy. Topotecan is a fluorescent drug and it dyes the living tissue. Then, it is recorded in a sequence of images over a period of time. The effective penetration of the drug depends on culture characteristics and requires a very specific timing which is calculated empirically by an expert. The purpose of this work is to offer a model to automatically estimate and evaluate the penetration time of topotecan in a cell, based on the information obtained from a sequence of tumorsphere images and using Shannon entropy and coefficient of variation.

Purpose: To describe a rare presentation of a case of intraocular non-teratoid medulloepithelioma with teratoid metastases in ipsilateral intraparotid lymph nodes. Case description: A 9-year-old male child with previous history of ciliary body non-teratoid medulloepithelioma presented with a swelling in the right pre-auricular region for 1 month. Magnetic resonance imaging and positron emission tomography–computed tomography showed a right intraparotid mass with enlarged ipsilateral cervical lymph nodes. A core biopsy was taken from the lesion, which on microscopy showed a tumor composed of small round cells arranged in cords, tubules lined by multilayered cells, and in cribriform pattern. These cells were embedded in a hypocellular, loose myxoid matrix. Based on the histopathological characteristics and previous history, a diagnosis of medulloepithelioma metastastic to ipsilateral parotid gland was made. The patient underwent right total conservative parotidectomy and bilateral neck dissection. Histopathological examination revealed metastatic medulloepithelioma in five out of eight intraparotid lymph nodes, with extranodal extension into the adjacent parotid parenchyma. Foci of hyaline cartilage were identified within the tumor, leading to a diagnosis of metastatic teratoid medulloepithelioma. Conclusion: Intraparotid lymph node metastases from intraocular medulloepithelioma is a rare possibility and we recommend that the parotid should be evaluated in cases of intraocular medulloepithelioma at initial presentation as well as during the follow-up period. Also, metastasis should be considered in all pediatric patients with solitary mass lesions showing unconventional histology for a primary parotid neoplasm.

Letter to the editor

Retinoblastoma is the most common intraocular tumor in childhood. It is not yet clear who the accompanying family members of affected children and teenagers are, how they find out about the disease, or how satisfied they are with the guidance provided by healthcare providers. Thus, this study aimed to portray the family members of children and teenagers with retinoblastoma regarding their sociodemographic aspects, the sources of information used by them to learn about the disease, their satisfaction with the guidance received from healthcare providers regarding this form of cancer, and to analyze whether there is an association between these variables. It was a cross-sectional, descriptive-analytical study, via web survey in Brazil, based on social media and analysis by descriptive-inferential statistics. A total of 129 people participated in this study, with an average age of 34.61 years, most of them were mothers, housewives, with eleven years of education, with a family income not exceeding minimum wage, and living mostly in the southeastern region of Brazil. Among the respondents, 65.12% of them stated that they use websites as their main source of information. Regarding the satisfaction with the guidance received from healthcare providers, there were scores above eight, indicating a high level of satisfaction with the instructional activity of physicians and nurses. It is concluded, in addition to the sociodemographic portrayal achieved, that websites emerged as the main source of information on retinoblastoma and that there was high satisfaction regarding the guidance received from healthcare providers. There was an association between family members whose occupation was considered to be that of a housewife and the use of social media as the main source of information regarding retinoblastoma, as well as an association between being self-employed and the satisfaction with the guidance provided by physicians regarding the disease.

Adenoma of the non-pigmented ciliary epithelium (NPCE) is a rare intraocular tumor which is often misinterpreted as a melanoma of the ciliary body or a non-tumorous process. We present a complex clinical, instrumental and morphological analysis of a case of adenoma of the NPCE growing into the base of the iris and the anterior chamber angle in a 13-year old child. Surgical treatment (iridocyclectomy) was performed. An adenoma of the NPCE was confirmed both histologically and by immunohistochemistry. At 6 months after surgery the patient's visual acuity was 1.0, with no signs of the tumor recurrence. The case can help to better understand clinical manifestation, results of the instrumental diagnostic assessments and the morphological picture of adenoma of NPCE that will be useful in the complex diagnosis of various intraocular tumors.

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., Video abstract

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.

Choroidal osteoma (CO) is a rare, benign, and usually unilateral intraocular tumor composed of mature bone affecting the choroid. It appears as slightly elevated, yellowish-white, or orange choroidal mass with well-defined borders. It is commonly encountered in young female adults. Here, we report findings of a 4-week-old premature baby with CO in her left eye which was detected during a routine examination for "retinopathy of prematurity." We believe that this case is the youngest patient reported with CO which showing that this pathology can be encountered even in a newborn.

Purpose To report an innovative new treatment option for ciliary body tumor metastasis from renal cell carcinoma treated with intravitreal bevacizumab. Observations A 70-year-old Caucasian man presented with a ciliary body tumor as a result from metastasis from renal cell carcinoma. It was preferable to treat the ciliary body tumor in the least invasive manner possible based on the patient's health, systemic metastasis, and the invasiveness of local resection. The tumor was treated with intravitreal bevacizumab for attempted tumor regression. Complete tumor resolution occurred following three injections. Conclusions and importance There is no consensus on treatment for ciliary body metastases from renal cell carcinoma. Based on the successful result of our patient, intravitreal bevacizumab could be an acceptable treatment option for this type of intraocular tumor. It affords a relatively non-invasive method of tumor regression without undergoing major intraocular surgery.

In the United States and other developed nations, more patients with retinoblastoma will die from non-ocular second malignant neoplasms than from their primary intraocular tumor. There is an increased lifetime risk of the development of second malignant neoplasms in survivors of heritable retinoblastoma compared with their nonheritable counterparts. Radiation increases the risk of second malignant neoplasms and should be avoided when possible. All patients at risk must be educated to remain vigilant for future signs or symptoms of malignancies and counseled to avoid exposure to other mutagens. The prognosis for patients with second malignant neoplasms is guarded and underscores the importance of counseling patients with the heritable form of the disease.

The main topic of this chapter is brachytherapy for ocular tumor. A small dish-shaped applicator is temporarily attached outside of the eyeball where the tumor is located, and after adequate irradiation time, it is removed. Because generally eyeball moves quickly and frequently, it is difficult to irradiate intraocular tumor with external beam technique. When big dose is delivered with adequate margin to compensate the organ motion, it is difficult to preserve visual function. On the other hand, when the applicator is accurately attached to the location of the tumor, the applicator moves together with the eyeball, and it is possible to perform very conformal irradiation. Therefore, ocular plaque brachytherapy is very important for the management of ocular tumors such as uveal melanoma and retinoblastoma.

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.

Retinoblastoma (RB) is the most common primary malignant intraocular tumor of childhood presenting usually before 5 years of age. RB in adults older than 20 years is extremely rare. A literature search using PubMed/PubMed Central, Scopus, Google Scholar, EMBASE, and Cochrane databases revealed only 45 cases till date. Over the past decade, there has been a significant increase in the number of such reports, indicating heightened level of suspicion among ophthalmologists. Compared to its pediatric counterpart, adult onset RB poses unique challenges in diagnosis and treatment. This article summarizes available literature on adult onset RB and its clinical and pathologic profile, genetics, association with retinocytoma, diagnostics, treatment, and outcomes.

Retinoblastoma is the most common intraocular tumor of childhood and can be either unilateral or bilateral and either hereditary or non-hereditary. Retinoblastoma is associated with mutations of the Rb gene on chromosome 13q, and chromosome 13q deletions and translocations in association with retinoblastoma have been described. Retinoblastoma can be associated with developmental delay, growth retardation, and congenital malformations. The authors describe a child with congenital hypotonia who developed retinoblastoma and was found to have a t(X;13) chromosomal translocation. The occurrence of hypotonia in association with retinoblastoma suggests an etiological link between the two disorders.

Retinoblastoma is a malignant intraocular tumor that mainly affects infants and young children. The diagnosis is made at an average age of 2 years for unilateral forms and 1 year for bilateral cases. The aim of our study was to report the atypical observation of a child with a unilateral endophytic retinoblastoma diagnosed at age 14.

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.

Background To present our experience on orbital and periorbital tissue changes after proton beam radiation therapy (PBRT) in patients with intraocular tumors, apart from treatment outcomes and disease control. Methods Medical records of 6 patients with intraocular tumors who had been treated with PBRT and referred to oculoplasty clinics of two medical centers (Seoul National University Hospital and Seoul Metropolitan Government-Seoul National University Boramae Medical Center) from October 2007 to September 2014 were retrospectively reviewed. The types of adverse effects associated with PBRT, their management, and progression were analyzed. In anophthalmic patients who eventually underwent enucleation after PBRT due to disease progression, orbital volume (OV) was assessed from magnetic resonance (MR) images using the Pinnacle3 program. Results Among the six patients with PBRT history, three had uveal melanoma, and three children had retinoblastoma. Two eyes were treated with PBRT only, while the other four eyes ultimately underwent enucleation. Two eyes with PBRT only suffered from radiation dermatitis and intractable epiphora due to canaliculitis or punctal obstruction. All four anophthalmic patients showed severe enophthalmic features with periorbital hollowness. OV analysis showed that the difference between both orbits was less than 0.1 cm before enucleation, but increased to more than 2 cm3 after enucleation. Conclusion PBRT for intraocular tumors can induce various orbital and periorbital tissue changes. More specifically, when enucleation is performed after PBRT due to disease progression, significant enophthalmos and OV decrease can develop and can cause poor facial cosmesis as treatment sequelae., Graphical Abstract

The following chapter describes step-by-step the surgical management of an intraocular tumor biopsy. The chapter contains the surgical management of a transretinal tumor biopsy, a transscleral tumor biopsy, an anterior chamber tumor biopsy, a vitreous biopsy, a chorioretinal biopsy, and last but least an intraocular biopsy in suspected lymphoproliferative disease.

Retinoblastoma the most common primary malignant intraocular tumor of childhood has been previously associated with neovascularisation of the iris and retina New vessel formation in the crystalline lens is an extremely rare entity in association with retinoblastoma In the present case we describe such a case with new rosettes and histopathological high risk factors with detailed pathological and histological documentations