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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.

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. 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.

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.

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.

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.

Melanocytoma or magnocellular nevus is a distinctive type of nevus that may originate on optic nerve head, choroid, ciliary body, iris. Melanocytoma of the optic disc is a pigmented lesion, occurring on the optic nerve head and often extends into the peripapillary retina and choroid. It may be sometimes difficult to distinguish between a melanocytoma and a malignant melanoma in the early stage. Although these tumors may grow slowly and invade surrounding ocular tissues, behaviourally they are benign. Ultrasonography is usually not helpful and fluorescein angiography can also often be misleading. Some unfortunate patients get enucleated due to lack of investigational support. We hereby present a case where diagnosis could be confirmed only on histopathological report of the optic nerve section following enucleation.

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.

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.

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.

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.

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

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.

Imaging the intraocular tumor is multi-modal, multi-purposed, evolving and essential in the detection, diagnosis and surveillance of intraocular tumors. The most useful modalities for imaging intraocular tumors are drawing, photography, ultrasound and optical coherence tomography. A primary purpose of imaging the intraocular tumor is to document its precise appearance (and that of its secondary effects) at a point in time, thus permitting sequential images to accurately document change. However, an equally important reason for imaging is to gather diagnostic information that cannot be obtained from ophthalmic examination alone. The specific utility of each available imaging modality depends on the patient, tumor and ability of the clinician to interpret the data produced.

In experiment on fresh enucleated eyes with large tumors it was shown that a new method of electrochemical lysis with original combined positioning of electrodes for intraocular tumors treatment provides a minimal traumatic impact and total destruction of tumor tissue at electrodes encirclement. Superficial electrode allows to aim the electrochemical destruction at hole tumor basis. the combination of the both superficial and intratumoral electrodes gives an opportunity to minimize a scleral injury in tumor basis scleral projection. Further examination on alteration of both intratumoral electrode injection depth and quantity of electricity in combinationwith bioimpedancemetry might it possible for regulation of morphological changes in tumor structure.

Purpose: to design the new combined technique of endoresection with intraoperative intraocular electrochemical lysis at the tumor destruction stage for large posterior intraocular tumors.Methods: 3 patients (3 eyes) with large choroidal melanomas t3N0M0 (tumor thickness — 8-10 mm, base diameter — 13-15 mm, juxtapapillary localization). Mean age was 55.4 years old. Endoresection with intraoperational intraocular electrochemical lysis of the tumor was performed. Electrochemical lysis was performed with use of the technical unit ECU 300 (Soering, Germany) and the original method of combined intratumoral positioning of two platinum electrodes: anode and cathode.Results: the tumor was removal completely in all 3 cases. the anatomical retinal reattachment was reached in all patients. Sclera was safe in all 3 cases. Visual acuity was not changed (NLP). At the place of the removal tumor a surgical choroidal coloboma without pigmentation all over scleral bed and periphery was shown in all cases in distant postoperative period (from 1.5 to 3 years). No local recurrences or metastasis were revealed in all patients.Conclusion: Further investigations in clinical group are necessarily to determinate the real possibilities of the combined method and the indications for endoresection with intraoperative intraocular electrochemical lysis for large intraocular tumors.

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

Choroidal nevus is the most common type of intraocular tumor with an estimated prevalence of 6.5% and 1.4% in Caucasian and Asian populations, respectively [1]. Due to pigmentation, melanotic choroidal nevi are relatively opaque to internal examination under white light. Operating at infrared wavelengths, swept source optical coherence tomography penetrates deeper into pigmented tissue and in the case of melanotic choroidal nevi can demonstrate internal features of nevi, such as blood vessels and granularity, as well as adjacent choriocapillaris abnormalities.

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)

Purpose The aim of this article is to report a case of uveal tuberculoma simulating an intraocular tumor in which the diagnosis was only possible by polymerase chain reaction (PCR) analysis. Method This is a case report. Results A36-year-old male presented with a progressive growing intraocular tumor and no history or positive test for other systemic disease. The eye eventually turned blind and painful and was enucleated. Histopathologic analysis revealed a granulomatous reaction and caseation necrosis but failed to identify any causative microorganisms. Final tuberculosis diagnosis was only possible by quantitative PCR. Conclusion Isolated uveal tuberculoma can present in an otherwise healthy patient with negative systemic tuberculosis evaluation. PCR can be used to confirm tuberculosis when other methods have failed.

Diffraction enhanced imaging (DEI) with edge enhancement is suitable for the observation of weakly absorbing objects. The potential ability of the DEI was explored for displaying the microanatomy and pathology of human eyeball in this work. The images of surgical specimens from malignant intraocular tumor of hospitalized patients were taken using the hard X-rays from the topography station of Beamline 4W1A at Beijing Synchrotron Radiation Facility (BSRF). The obtained radiographic images were analyzed in correlation with those of pathology. The results show that the anatomic and pathologic details of intraocular tumors in human beings can be observed clearly by DEI for the first time, with good visualization of the microscopic details of eyeball ring such as sclera, choroids and other details of intraocular organelles. And the best resolution of DEI images reaches up to the magnitude of several tens of μm. The results suggest that it is capable of exhibiting clearly the details of intraocular tumor using DEI method.

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.

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.

Retinoblastoma is the most common primary malignant intraocular tumor of childhood. It may present with atypical features, particularly in older children. The authors report a case of unsuspected retinoblastoma following evisceration in a 9-year-old girl who had previous pars plana vitrectomy in the affected eye.

Uveal melanoma is the most frequent primary malignant intraocular tumor in adults. Secondary glaucoma is a rare and unusual circumstance discovery of a malignant choroidal melanoma. We report through a case report, clinical signs, management and prognosis of acute angle glaucoma as initial manifestation of uveal melanoma.

Autofluorescence is a relatively new, noninvasive diagnostic tool recently introduced in the armamentarium of intraocular tumor diagnosis and characterization. This technique may reveal the effects of choroidal tumors on the overlying retinal pigment epithelium and may also provide specific information on intrinsic pigments that compose and characterize benign and malignant, congenital and acquired chorioretinal lesions. Lipofuscin, melanin, and melanolipofuscin strongly influence the different autofluorescence patterns of chorioretinal tumors, but other pigments may theoretically influence the autofluorescence pattern of these lesions. In this chapter we review the role of autofluorescence in the diagnosis of benign and malignant chorioretinal.

7 p., 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.

1543Background: Uveal melanoma (UM) is the most common primary intraocular tumor in adult, but its etiology for the most part remains unclear. It has been suggested that the genetic susceptibility ...

The ability to obtain the proper clinical diagnosis in cases of suspected intraocular tumors has greatly advanced during the past 50 years. The clinical characteristics of most intraocular tumors (size, shape, color, and texture) are detectable by skilled ophthalmoscopic examination and, with the use of adjunctive techniques (mainly ultrasonography), the proper diagnosis can be reached without invasive procedures. Notwithstanding, some intraocular tumors need to be biopsied to obtain a reliable diagnosis. In the cytogenetic era, intraocular tumor management is changing, and tumor-sampling procedures are becoming the main prognostic (and theoretically also diagnostic) tools for uveal melanoma. In spite of the widespread use of biopsies in general surgical practice, in ophthalmic oncology, indications and contraindications for biopsy continue to be under debate. The purpose of this paper is to critically evaluate the role of uveal melanoma biopsy in current clinical practice.

Introduction: Intra-arterial chemotherapy (chemosurgery) for the treatment of retinoblastoma has been performed more than 1600 times (more than 1400 times in Japan and 200 times in New York) over the past 20 years. Despite this treatment's success some eyes cannot be saved and require enucleation. Here we report the histopathologic findings of the remaining intraocular tumor of eyes that were enucleated following treatment that included chemosurgery in New York City. Materials and Methodology: Independent histopathologic review of the enucleated eyes was correlated with the clinical findings that prompted enucleation. Results: Between May 1, 2006 and April 30, 2009, 56 eyes received chemosurgery at our institution, and 10 of these were enucleated subsequently. All were Reese Ellsworth Group 5 at enucleation. Of the 21 eyes that were treated with chemosurgery as the primary treatment, 1 (5%) was enucleated subsequently; its histopathology revealed residual non- necrotic, non-calcified tumor. Of the 34 eyes treated with chemosurgery after other treatments, 9 (24%) were enucleated, and 5 of these eyes contained non-calcified, non-necrotic tumor. None was enucleated for complications of chemosurgery. All patients were alive and free of metastatic disease as of September 2009. Conclusions: A significant number of eyes with advanced intraocular retinoblastoma avoided enucleation as a result of chemosurgery. The rate of eyes that were enucleated was higher when chemosurgery was the secondary rather than the primary treatment. Of the eight eyes enucleated for progressive disease six had non-necrotic, non-calcified tumor cells.

PURPOSE: To describe the first case of intraocular teratoma associated with eyelid coloboma and the second reported case of intraocular teratoma. DESIGN: Interventional case report. METHODS: A left intraocular tumor was surgically resected from a 2-day-old female with an associated lower eyelid coloboma. RESULTS: Pathologic evaluation revealed a completely intraocular tumor comprising derivatives of all three germ cell layers giving a diagnosis of intraocular teratoma. The eyelid coloboma was repaired, and a scleral-wrapped hydoxyapatite-integrated orbital implant was placed. CONCLUSION: To our knowledge, this is the second reported instance of teratoma originating within the globe and the only reported case of teratoma associated with eyelid coloboma. Although exceedingly rare, intraocular teratoma should be added to the differential diagnosis of congenital intraocular tumors.

e20054 Background: Uveal melanoma is the most common primary intraocular tumor in adults, median age at diagnosis of 60 years. Despite a growing understanding of the molecular characteristics of uv...