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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.

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.

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

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.

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.

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.

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.

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.

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.

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

Understanding the mechanism of retinoblastoma (Rb) tumor initiation, development, progression and metastasis in vivo mandates the use of animal models that mimic this intraocular tumor in its genetic, anatomic, histologic and ultrastructural features. An early setback for developing mouse Rb models was that Rb mutations did not cause tumorigenesis in murine retinas. Subsequently, the discovery that the p107 protein takes over the role of pRb in mice led to the development of several animal models that phenotypically and histologically resemble the human form. This paper summarizes the transgenic models that have been developed over the last three decades.

Uveal melanoma (UM) is the most frequent primary intraocular tumor in Caucasian adults and is potentially fatal if metastases develop. While several prognostic genetic changes have been identified in UM, epigenetic influences are now getting closer attention. Recent technological advances have allowed to examine the human genome to a greater extent and have improved our understanding of several diseases including malignant tumors. In this context, there has been tremendous progress in the field of UM pathogenesis. Herein, we review the literature with emphasis on genetic alterations, epigenetic modifications and signaling pathways as well as possible biomarkers in UM. In addition, different research models for UM are discussed. New insights and major challenges are outlined in order to outline the current status for this potentially devastating disease.

Background: The objective of this study is to evaluate the risk of optic nerve invasion associated with optic nerve obscuration at diagnosis or persisting during treatment. Methods: Retrospective review from 2011-2016 of patients with advanced retinoblastoma (Group D/E) with complete obscuration of the nerve at diagnosis and a second group of patients with persistent, complete obscuration throughout treatment. Results: Advanced retinoblastoma was diagnosed in 102 eyes of 86 patients. The optic nerve was obscured in 69 eyes (68%) at diagnosis. Of these, 30 (43%) underwent salvage therapy and 39 (57%) primary enucleation. Histopathologic analysis of primarily enucleated eyes showed 41% prelaminar and 15% postlaminar invasion. Four eyes in the salvage group demonstrated persistent nerve obscuration; 2 were subsequently enucleated without evidence of nerve invasion. Average follow-up was 23.5 months (range 1-62 months). Conclusions and Relevance: Optic nerve obscuration at diagnosis may be associated with postlaminar optic nerve invasion. While persistent, complete obscuration of the optic nerve by retinoblastoma during treatment is a poor prognostic sign for both globe salvage and vision, it does not appear, in this small cohort, to increase the risk of optic nerve invasion. With appropriate control of the intraocular tumor, these eyes can be salvaged.

The pediatric malignancy retinoblastoma is the most common intraocular tumor in the world, reflecting the rarity of uveal melanoma in populous nations like China and India.1,2 Kivela has estimated that there are approximately 8000 retinoblastomas yearly in the world and about 1000 fewer uveal melanomas, which typically occur in older adults.2

Uveal melanoma (UM) is a malignant intraocular tumor in adults. Metastasis develops in almost half of the patients and over 90% of the metastases are in the liver. With the advances in molecular targeting therapy for melanoma, a proper metastasis animal model is of increasing importance for testing the accuracy and effectiveness of systemic therapies. Here, we describe a xenograft model for mimicking human UM liver metastasis by injecting human UM cells into the vitreous cavity in nude mice. The athymic nude mice are immunocompromised and suitable for xenograft tumor growth and metastasis, and intravitreal injection of cells is a quicker and easier operation under a binocular scope, thereby it is simple and effective to test human UM growth and metastasis.

The patients’ survival for the most common intraocular tumor in children, retinoblastoma, has a wide spectrum among the world countries. This study was conducted to provide an overview of the retinoblastoma survival disparity worldwide by discussing the trends of patients’ survival, as well as recent advances in the management of retinoblastoma.The design of this study was literature review and commentary. Selected articles from PubMed (except one) including both developing and developed countries regarding the patients’ survival in retinoblastoma were considered and reviewed critically. An analysis of 47 articles was performed. In conclusion, in spite of an obvious contrast of retinoblastoma survival disparity in the world, the expanding horizons in developing countries are promising and continuing on all fronts and results are hope-inspiring.

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.

Malignant teratoid medulloepithelioma of ciliary body is an extremely rare tumor usually occurring in children younger than 5years of age and is usually amelanotic. Here, we report a very rare case of pigmented malignant teratoid medulloepithelioma in a 20-year old male patient who presented initially with pain, redness and proptosis of right eyeball. The right eyeball was enucleated. A histopathological diagnosis of orbital teratoma was made and no further treatment was given. Four months later, the patient developed another swelling in the same area. A review of the previous histopathology slides revealed a pigmented malignant teratoid medulloepithelioma. No nerve involvement was seen. MRI did not show any intracranial extension. The mass along with rest of the orbital tissue was removed. No adjuvant chemotherapy or radiotherapy was given due to the lack of intracranial extension. The patient was symptom free at 2-year follow-up and the repeat MRI also showed no evidence of recurrence.

The authors describe three cases of trilateral retinoblastoma, a rare syndrome that occurs most often in young children. Two of these children were 3 years old at diagnosis, and the other 4 years old. The retinoblastoma was unilateral in one child and bilateral in the others. All underwent computed tomography (CT) imaging, which made it possible to locate the intraocular tumor lesions, to specify the extent of the tumor and to look for a possible intracranial neoplasm. Unfortunately, the cost of CT is high, which limits its use in our context.

Eyes to be enucleated due to intraocular tumor or absolute glaucoma were used in an ultrasonic investigation of foreign bodies situated retrosclerally, or in the choroid-scleral complex. The echograph findings of four intra-choroidal, five intrascleral and three retroscleral foreign bodies were demonstrated. The foreign bodies were of iron, and were polygonal in shape. The intrachoroidal foreign bodies weighed 0.081–0.327 mg, the intrascleral 0.120–0.685 mg and the retroscleral 0.787–1.456 mg. In cases where double perforation is suspected, and where the ocular media are turbid, ultrasonography would appear capable of giving valuable information as to whether a foreign body is located intra- or extraocularly.

In this review, we discuss the genetic features of uveal melanoma and the different available genetic and genomic tests. Despite improvements in diagnosis and treatment of this intraocular tumor, there has not been a change in survival in the past few decades. Insights into the molecular changes in uveal melanoma have provided us with the opportunity to assess the risk of metastatic disease for individual patients. There are few treatments available for metastasized uveal melanoma and, in a relatively short time-frame, patients with this condition die because of this metastasis. The focus of research may need to shift towards adjuvant therapies for high-risk uveal melanomas.

Background Retinoblastoma is a malignant tumor of the embryonic neural retina. About 80% of cases are diagnosed before age 4, with a median age at diagnosis of 2 years. Objective To determine characteristics and prognosis of retinoblastoma in children older than 5 years. Procedures From 1986 to 2002, medical records of 16 patients out of 453 cases referred to Hospital do Câncer AC Camargo, Sao Paulo, Brazil. Results Median age at diagnosis was 73.7 months (range 65–144) and there was an equal gender distribution. Fifteen patients presented with unilateral disease. The mean time between first symptoms and diagnosis was 9.6 months (range 0–48). Most cases were diagnosed in advanced stages and 15 eyes were enucleated. Eleven patients presented with intraocular tumor (1 Reese II and 10 Reese V) and five presented with extraocular disease (one CCG II and four CCG III). Twelve patients are still alive with a median follow-up of 92 months (range 65–199). Conclusions Because of its low incidence at this age, diagnosis of retinoblastoma is usually delayed due to low level of suspicion. Therefore, it is important that physicians are aware of this disease in order to perform an earlier diagnosis, and decrease treatment-related morbidity. Pediatr Blood Cancer 2007;48:292–295. © 2006 Wiley-Liss, Inc.

Uveal melanoma is a malignant intraocular tumor associated with poor vital prognosis, process generalization, and death from metastatic disease. A number of factors are considered undesirable in the presence of a tumor. This paper provides a review of the literature on major prognostic factors for uveal melanoma development and their contribution to vital prognosis.Увеальная меланома - злокачественная внутриглазная опухоль с плохим витальным прогнозом, приводящая к генерализации процесса и смерти больных от метастатической болезни. Существует ряд факторов, оказывающих неблагоприятное воздействие на течение опухолевого процесса. Настоящая работа представляет обзор литературы по основным прогностическим критериям увеальной меланомы и их значимости для витального прогноза заболевания.

The molecular and cellular basis of human choroidal malignant melanoma progression has remained largely unknown. However, choroidal melanoma is the most important primary intraocular tumor in adults. Developmentally, choroidal melanocytes are of neural crest origin similar to cutaneous melanocytes. However, there are some significant differences between cutaneous and uveal melanocytes that have yet to be fully assessed. The purpose of this study is to describe choroidal melanocytes. We will describe the significant differences between cutaneous and uveal melanocytes as well as the congenital and acquired diseases of uveal melanocytes. We will then describe the cellular and molecular mechanisms involved in melanoma progression.

Primary uveal melanoma is extremely rare in children and also among blacks. Uveal melanomas may be associated with various preexisting conditions, including congenital ocular melanocytosis and dysplastic nevus syndrome. We report the case of a 4-year-old black girl who presented with pronounced proptosis and no light perception secondary to a massive intraocular tumor with extrascleral extension. Biopsy of the tumor confirmed the diagnosis of primary uveal melanoma, and the patient was treated by enucleation and external beam radiation therapy. Although metastatic disease was ruled out at the time, the patient died approximately 3 months later.

To present the case of a patient with leiomyoma of the ciliary body and discuss the histological features and treatment of this rare intraocular tumor. METHODS/CASE REPORT: A 13-year-old boy presented with an asymptomatic tumor of the right eye. Visual acuity was 20/20 in both eyes. Ophthalmoscopy revealed an amelanotic, vascularized ciliary body tumor with exudative retinal detachment and partial transillumination. On ultrasound examination the tumor height was 8 mm and a low internal reflectivity was found. T2-weighted MRI scans showed a hypointense and T1-weighted scans a hyperintense intraocular mass with significant Gd-TPA enhancement. On the assumption that the diagnosis was consistent with an amelanotic ciliary body melanoma, a transscleral resection with adjuvant ruthenium-106 brachytherapy was performed. Visual acuity was 20/40 at 6 months after the operation.Routine stains revealed a pleomorphic tumor composed mainly of spindle cells with palisading in some areas and a prominent intercellular fibrillary background. Immunohistochemistry showed positivity for desmin, vimentin and actin. No reactivity with S-100 and HMB-45 was seen. Intracytoplasmatic filaments and micropinocytotic vesicles were detected by transmission electron microscopy. These findings were consistent with the diagnosis of a ciliary body leiomyoma.Typical clinical features of leiomyoma include a dome-shaped configuration and translucency, but the final diagnosis can only be confirmed by histology with the aid of immunohistochemistry and electron microscopy. Though rare, leiomyoma should be considered in the differential diagnosis of amelanotic uveal tumors. Transscleral resection is the treatment of choice of anterior uveal leiomyomas, with a fairly good visual prognosis.