In a pickle: Cases of pickleball related ocular injuries.

Purpose: Although musculoskeletal involvement is the common presentation, studies have reported the incidence of sports related ocular trauma. Here we present the case reports of two patients who sustained injury in one of the fast growing sports - the pickleball, during play without eye protection. Observations: Two patients with history of injury during pickleball play presented to our clinic with varying spectrum of ocular presentations. First patient had an anterior segment involvement with hyphema and elevated intraocular pressure, and the second patient had both anterior and posterior segment involvement causing angle recession and commotio retinae. Conclusion: Ocular injuries related to various sports have been extensively described, our intent is to increase the awareness about the possible ocular injuries related to rising pickleball and that improved safety measures and appropriate education to the players could prevent such ocular injuries.

Multimodal imaging risk factors predictive of small choroidal melanocytic lesion growth to melanoma: An educational study and pictorial guide.

BACKGROUND: Risk factors for small choroidal melanocytic lesion growth to melanoma have been redefined using multimodal imaging. We explored provider ability to recognize risk factors for small choroidal melanocytic lesion growth to melanoma before and after image-based education and with and without multimodal imaging. METHODS: Providers were invited to participate in a survey assessing ability to identify risk factors for small choroidal melanocytic lesion growth to melanoma using either fundus imaging or multimodal imaging. Risk factors included thickness >2 mm on ultrasonography, subretinal fluid on optical coherence tomography, presence of orange pigment by autofluorescence, acoustic hollowness by ultrasonography, and diameter >5 mm by fundus imaging. Performance was assessed before and after reviewing an educational PowerPoint providing pictorial examples of risk factors. Comparison between groups was conducted using two-tailed Fisher's exact test. RESULTS: Thirty and 26 providers completed the pre-education and post-education assessments, respectively. Post-education participants were more accurate within ±1 risk factor for lesions with zero risk factors (77% vs. 100%, p = 0.01) or two risk factors (79% vs. 91%, p = 0.03). Following education, participants presented with multimodal imaging more often correctly identified lesions with four (12% vs. 42%, p = 0.03) or five (4% vs. 39%, p = 0.004) risk factors, demonstrated lower mean level of concern for lesions with zero risk factors (2.0 vs. 1.4, p < 0.001), and expressed higher level of concern for lesions with 5 risk factors (2.4 vs. 3.6, p < 0.001). CONCLUSION: Use of multimodal imaging may be more beneficial than education itself to improve accuracy of risk factor identification for small choroidal melanocytic lesions.

Malignant Epiretinal Membrane After Proton Beam Radiation of Ciliary Body Melanoma.

Purpose: To report the development of malignant epiretinal membrane after radiation of ciliary body melanoma. Case Report: A 65-year-old woman was referred for evaluation of a ciliary body tumor in her right eye. On examination, a pigmented ciliary body tumor, displacing the iris anteriorly, was visible superotemporally and ultrasound biomicroscopy revealed a large solid ciliary body tumor. She was diagnosed with ciliary body melanoma and treated with proton beam radiation. Over the following 29 months, the treated tumor regressed but optical coherence tomography (OCT) showed the development of a dense epiretinal membrane. Enucleation was performed and histopathological examination showed viable melanoma cells in the vitreous cavity with sheet-like growth of viable spindle melanoma cells on the epiretinal surface. Conclusion: The development of a pigmented epiretinal membrane in eyes with uveal melanoma should raise the possibility of a malignant epiretinal membrane.

Diagnosing Vitreoretinal Lymphomas-An Analysis of the Sensitivity of Existing Tools.

Vitreoretinal lymphoma (VRL) is a rare ocular pathology that is notorious for mimicking chronic uveitis, which is a seemingly benign condition in comparison. The most common form of VRL is the diffuse large B-cell type, and there has been a high mortality rate. This dismal prognosis can be improved significantly if the disease is diagnosed early, but until now there is no consensus on an appropriate diagnostic algorithm. We conducted a retrospective search of PubMed Central® and analyzed results from thirty-three studies that were published between 2011-2021. The chosen studies incorporated some popular testing tools for VRL, and our analyses focused on comparing the average sensitivity of five diagnostic methods. The methods included cytology including ancillary immunohistochemistry, Myeloid Differentiation Factor 88 (MyD88) mutation analysis, polymerase chain reaction (PCR) for monoclonal rearrangements of immunoglobulin heavy chain (IgH) and T-cell Receptor (TCR) genes, flow cytometry, and IL10 and IL6 analysis. Across the varied diagnostic methods employed in thirty-three studies explored in this analysis, MyD88 mutation assay emerged as a strong contender given its sensitivity and low coefficient of variation. There is an imminent need for the introduction of newer assays that can further improve the sensitivity of identifying MyD88 mutation in cancer cells seen in the vitreous.

DIAGNOSTIC CHALLENGES IN NECROTIC UVEAL MELANOMA.

PURPOSE: To describe a case of ciliochoroidal melanoma with massive spontaneous hemorrhagic retinal and choroidal detachment that presented a diagnostic challenge because of poor fundus visualization and atypical imaging features. METHODS: Case report. RESULTS: A 71-year-old white man on apixaban for atrial fibrillation presented with sudden onset of pain and vision loss in the left eye. The patient was found to have total hemorrhagic retinal detachment and was referred for our opinion. On examination, visual acuity was 20/25 in the right eye and light perception in the left eye. Intraocular pressures were 10 mmHg in the right eye and 21 mmHg in the left eye. The unaffected right eye demonstrated only macular retinal pigment epithelial alterations. Evaluation of the left eye revealed dilated episcleral sentinel vessels superonasally, shallow anterior chamber, and total hemorrhagic retinal and choroidal detachment to the back of the crystalline lens. There was no view of the choroid. Transillumination demonstrated diffuse blockage of light from blood and no specific shadow. B-scan ultrasonography revealed total retinal and choroidal detachment with dense echoes of blood under the retina and in the suprachoroidal space with elevation of 12 mm and no definite mass or spontaneous vascular pulsations. Magnetic resonance imaging revealed diffuse high signals within the globe on T1-weighted images with no specific mass. On gadolinium enhancement, a localized enhancing area temporally of 12-mm thickness and a localized nonenhanced area nasally of 11-mm thickness was noted. Both areas showed low signal on T2-weighted images. Despite atypical features, suspicion for underlying ciliochoroidal melanoma and poor visual prognosis lead to enucleation. Histopathology confirmed an extensively necrotic, mushroom-shaped ciliochoroidal melanoma with total hemorrhagic retinal and choroidal detachment. CONCLUSION: Diagnosis of necrotic posterior uveal melanoma can be challenging, because these tumors can be camouflaged by overlying hemorrhagic retinal and choroidal detachment and can present with atypical features on ultrasonography and magnetic resonance imaging. Patients with spontaneous hemorrhagic retinal and choroidal detachment should be evaluated for possible underlying malignancy.

CHOROIDAL NEOVASCULAR MEMBRANE AFTER FINE-NEEDLE ASPIRATION BIOPSY OF VITREORETINAL LYMPHOMA.

PURPOSE: To report development of choroidal neovascular membrane at the site of diagnostic transvitreal fine-needle aspiration biopsy of subretinal pigment epithelial infiltrates in an eye with vitreoretinal lymphoma. METHODS: Case report. A 75-year-old white woman with 14-month history of bilateral vitritis. RESULTS: Examination showed vitreous infiltration in both eyes and yellow-white subretinal pigment epithelial infiltrates temporally in the left eye. Visual acuity was 20/400 in both eyes. Transvitreal fine-needle aspiration biopsy of subretinal pigment epithelial infiltrate in the left eye using a long 27-gauge needle attached to a 10-mL syringe revealed large B-cell lymphoma. Treatment was initiated with monthly intravitreal injections of melphalan (10 µg/0.05 mL) to both eyes. One month after first injection, visual acuity in the left eye had decreased to hand motion, and new ill-defined patches of retinal whitening were noticeable in the temporal macular area. Intravenous fluorescein angiography and optical coherence tomography angiography showed large choroidal neovascular membrane temporally at the site of previous fine-needle aspiration biopsy. Four monthly injections of intravitreal bevacizumab (1.25 mg/0.05 mL) resulted in complete regression of choroidal neovascular membrane with improvement of visual acuity to 20/100 in the left eye. CONCLUSION: This case demonstrates the rare development of choroidal neovascular membrane at the site of diagnostic transvitreal fine-needle aspiration biopsy of subretinal pigment epithelial infiltrates in an eye with vitreoretinal lymphoma.

BAP1 GERMLINE MUTATION WITH IRIDOCILIOCHOROIDAL MELANOMA AND MALIGNANT PERITONEAL MESOTHELIOMA.

PURPOSE: BAP1 germline mutation can promote risk of uveal melanoma and mesothelioma. In this study, we report a patient with BAP1 germline mutation and uveal melanoma who developed a rare form of mesothelioma in the peritoneum. METHOD: Case report. RESULTS: A 55-year-old white man noted blurred vision in the left eye. Examination revealed superotemporal episcleral sentinel vessels and a dark brown iris mass suspicious for melanoma, with angle invasion and involvement of the ciliochoroidal region. The tumor measured 14 mm in diameter and 10.3 mm in thickness. A diagnosis of iridociliochoroidal melanoma was rendered and plaque radiotherapy applied. Genetic testing revealed BAP1 germline mutation. At 3.5-year follow-up, tumor control was achieved, but radiation-related complications led to enucleation. At 5.5-year follow-up, incidental findings by laparoscopy revealed multiple peritoneal tumors, histopathologically proven to be malignant peritoneal mesothelioma. Lung evaluation was normal, and there was no melanoma-related or mesothelioma-related metastasis. Likewise, magnetic resonance imaging of the abdomen showed no evidence of hepatic metastasis. CONCLUSION: BAP1 tumor predisposition syndrome can promote risk of uveal melanoma and malignant peritoneal mesothelioma. Cancer screening for these malignancies is warranted.

Photodynamic Therapy for Retinal Hemangioblastoma: Treatment Outcomes of 17 Consecutive Patients.

PURPOSE: To report the efficacy of photodynamic therapy (PDT) for management of retinal hemangioblastoma. DESIGN: Retrospective case series. PARTICIPANTS: Seventeen patients with retinal hemangioblastoma treated with PDT. METHODS: The medical records of 17 patients with retinal hemangioblastoma treated with PDT were reviewed, and treatment outcomes were assessed. Photodynamic therapy was performed with 6 mg/m2 body surface area of verteporfin infused intravenously over 10 minutes activated by 50 J/cm2 laser light at 689 nm for 83 or 166 seconds. MAIN OUTCOME MEASURES: Tumor control, subretinal and intraretinal fluid resolution, and visual outcome. RESULTS: Eighteen retinal hemangioblastomas in 17 eyes were treated with PDT. Median patient age was 31 years (mean, 36 years; range, 7-66 years), and median follow-up was 51 months (mean, 61 months; range, 2-144 months). Genetic testing confirmed von Hippel-Lindau disease in 8 of 17 patients (47%). The tumors were unilateral in all patients and unifocal in most patients (n = 13/17 [76%]). The tumor median basal diameter was 3.5 mm (mean, 3.4 mm; range, 1.5-6.0 mm) and median thickness was 2.1 mm (mean, 2.3 mm; range, 1.0-5.0 mm). The tumor location was juxtapapillary in 9 of 18 cases (50%). Associated findings included subretinal fluid (n = 14/17 [82%]) and macular edema (n = 12/17 [71%]). The median number of PDT sessions was 1.5 (mean, 1.8; range, 1.0-4.0). Standard duration of PDT (83 seconds) was used in all cases except 2, in which double duration (166 seconds) was used. Outcomes revealed tumor control in 13 of 18 tumors (72%), partial or complete resolution of subretinal fluid in 10 of 14 eyes (71%), and partial or complete resolution of macular edema in 7 of 12 eyes (58%), and stable or improved visual acuity in 12 of 17 eyes (71%). Photodynamic therapy-related transient exudative response was noted in 4 of 17 eyes (24%). CONCLUSIONS: Photodynamic therapy is an effective treatment for both juxtapapillary and peripheral retinal hemangioblastomas, providing satisfactory rates of tumor control and visual stabilization and improvement. Patients should be monitored for PDT-related transient exudative response.

Uveal Melanoma Metastasis to the Contralateral Eye Structures: A Retrospective Comparative Analysis of 13 Consecutive Patients.

PURPOSE: To report uveal melanoma (UM) metastasis to the contralateral ocular and periocular structures. DESIGN: Retrospective study. PARTICIPANTS: Thirteen patients with UM metastasis to the contralateral ocular and periocular structures were included. METHODS: Clinical records were reviewed retrospectively. MAIN OUTCOME MEASURES: The development and time to onset of contralateral ocular and periocular metastasis, systemic metastasis, and death. RESULTS: Of the 13 000 treated UM patients, 13 patients were diagnosed with UM metastasis to the contralateral ocular and periocular structures. Mean patient age at primary UM diagnosis was 60 years (median, 60 years; range, 37-87 years). The primary uveal melanoma was in the choroid (n = 11) or ciliary body (n = 2) and was treated with brachytherapy (n = 11), proton beam radiotherapy (n = 1), or enucleation (n = 1). Systemic metastasis developed in 11 patients (85%) at a mean of 66 months (median, 34 months; range, 12-216 months) after diagnosis of the primary UM. All 11 patients (100%) showed liver metastasis and 8 patients (62%) also showed extrahepatic metastasis. The sites of metastasis to the contralateral ocular or periocular structures included the choroid in 4 patients (31%), the orbit in 7 patients (54%), and the eyelid in 2 patients (15%). One patient with eyelid metastasis demonstrated concurrent conjunctival nodule. Mean time to diagnosis of contralateral ocular or periocular metastasis was 94 months (median, 48 months; range, 9-375 months). Contralateral choroidal metastasis was multifocal in 3 of 4 patients (75%). Of 7 patients with orbital metastasis, 5 showed extraocular muscle involvement with restricted ocular motility. Treatment for contralateral choroidal metastasis included brachytherapy (n = 2), transpupillary thermotherapy (n = 1), and observation (n = 1). Treatment for contralateral periocular (orbit or eyelid) metastasis was excision (n = 5), external beam radiotherapy (n = 2), and observation (n = 2). Of 13 patients, death was documented in 11 patients at a mean of 17 months (median, 9 months; range, 3-54 months) as a result of systemic UM metastasis (n = 10) or unrelated cause (n = 1). CONCLUSIONS: Metastasis resulting from UM to the contralateral ocular and periocular structures is rare and generally occurs in patients with disseminated metastasis. Orbital tissue is the most common site of involvement, and these patients have short life expectancy.

ASSOCIATION OF VITREORETINAL LYMPHOMA WITH SYSTEMIC LYMPHOMA.

PURPOSE: To determine the association between vitreoretinal lymphoma and systemic lymphoma (SL). METHODS: Single-center retrospective review of medical records. RESULTS: Of 95 patients with vitreoretinal lymphoma, 18 (19%) had associated SL (SL group) and 77 (81%) were not associated with SL (no SL group). The most common sites of SL were skin (n = 5), testis (n = 2), liver and breast (n = 2), and others (n = 9). A comparison (SL group vs. [vs.] no SL group) revealed no difference in demographic or ocular findings at initial visit. In the SL group, SL occurred before the onset of ocular symptoms in 14 (78%) patients with mean interval of 86 months (median 61, range 5-286 months) or after ocular symptoms in 4 (22%) patients with mean interval of 19 months (median 12, range 7-44 months). A comparison revealed no difference in overall frequency of pre-existing or eventual central nervous SL (50% vs. 53%, P = 0.99); however, the SL group demonstrated central nervous SL more often after onset of ocular symptoms (78% vs. 17%, P = 0.001). A comparison found no difference in treatment methods, response of vitreoretinal lymphoma to treatment, final visual outcome, or death rate. CONCLUSION: We found 19% of patients with vitreoretinal lymphoma demonstrate related SL, and there was no difference in demographics, clinical features, or response to treatment, compared to those not associated with SL.

GROWTH OF PRESUMED CHOROIDAL NEVUS INTO MELANOMA OVER 4 YEARS IN BAP1 TUMOR PREDISPOSITION SYNDROME.

PURPOSE: To report a case of presumed choroidal nevus that eventually grew into melanoma in a patient with family history of choroidal melanoma and germline BAP1 mutation. METHODS: Case report. RESULTS: A 55-year-old healthy white woman with a family history of uveal melanoma in her father, paternal aunt, and paternal cousin was referred for evaluation of an asymptomatic small pigmented choroidal lesion in her right eye, measuring 2 mm × 2 mm in basal diameter and 1 mm in thickness. There were no clinical risk factors. The patient was advised routine monitoring but returned 4 years later with intermittent photopsia. The choroidal mass demonstrated growth and suggestive of transformation into melanoma, measuring 9 mm × 6 mm in basal diameter and 2.5 mm in thickness with overlying orange lipofuscin pigment and no associated subretinal fluid. Fine-needle aspiration biopsy disclosed Chromosome 3 mosaic monosomy and Chromosomes 6 and 8 disomy. Iodine 125 plaque radiotherapy was provided. Based on growth to melanoma and strong family history of uveal melanoma, BAP1 germline mutation testing was performed, and the results were positive. CONCLUSION: This case demonstrates growth of a presumed choroidal nevus into melanoma in the setting of underlying germline BAP1 mutation. We suggest that small pigmented choroidal lesions be monitored closely in patients with germline BAP1 mutation or with family history of uveal melanoma, even in the absence of known local risk factors predictive of tumor growth.

MULTIMODAL IMAGING OF MACULAR CHOROIDAL MACROVESSEL: A REPORT OF TWO CASES.

PURPOSE: To report two cases of macular choroidal macrovessel with description of multimodal imaging and review of published cases. METHODS: Medical and imaging records were retrospectively reviewed. A literature review was performed to identify other cases of macular choroidal macrovessel published between 1990 and 2018. RESULTS: There were 2 patients referred for evaluation of a potential choroidal tumor, including a 55-year-old white woman with no visual symptoms and a 68-year-old white woman with blurred vision. Funduscopic examination in each case revealed a focal area of choroidal elevation in the temporal foveal area with a single, dilated, slightly tortuous choroidal vessel, estimated to measure 250 µm and 300 µm, respectively, at widest girth, and extending temporally toward the equator with a tapering width. Optical coherence tomography showed an optically hollow choroidal lesion elevating the retinal pigment epithelium with overlying ellipsoid zone mottling. Both cases demonstrate shallow subretinal fluid, sparing the foveola. Fluorescein angiography showed faint staining along the course of the vessel in both cases. Indocyanine green angiography demonstrated clear visualization of the ectatic vessel with early filling and late staining without leakage. Both patients retained visual acuity of 20/25 in the affected eye and did not require treatment. CONCLUSION: Macular choroidal macrovessel is a rare vascular anomaly presenting as a single, dilated, tortuous choroidal vessel, originating in the temporal foveal region and extending temporally to the equator. This finding can simulate a choroidal neoplasm or parasitic track. Optical coherence tomography and indocyanine green angiography have diagnostic value demonstrating the vascular nature of this lesion and delineating the vascular course.

INTRAVITREAL MELPHALAN FOR TREATMENT OF VITREOUS SEEDING FROM CHOROIDAL MELANOMA.

PURPOSE: To report treatment of vitreous seeding of choroidal melanoma with monthly injections of intravitreal melphalan. METHODS: Case report. RESULTS: A 70-year-old white woman noted floaters in her left eye, and further examination revealed visual acuity of 20/30 in both eyes. Funduscopically, there was a mushroom-shaped choroidal melanoma in her left eye, measuring 9 mm in basal dimension and 4.8 mm in thickness. Notably, there was apical retinal invasion of melanoma with mild vitreous hemorrhage, without vitreous seeding. The tumor was treated with iodine-125 plaque radiotherapy using an apex dose of 70 Gy over 99 hours, designed to include the retinal invasion. The melanoma demonstrated complete regression into a nearly flat scar of 1 mm and remained stable over 4 years. Five years after radiotherapy, there were diffuse vitreous pigmented seeds of presumed melanoma origin, emanating from the site of retinal necrosis. This progressively worsened over the following 18 months, suspicious for viable melanoma cells, as visual acuity concurrently declined to 20/100. Treatment with intravitreal melphalan (10 µg/0.05 mL) was delivered on a monthly basis for 12 cycles, resulting in vitreous seeds regression, and preservation of the eye. Final visual acuity was 20/200. There were no treatment-related complications. CONCLUSION: Intravitreal melphalan can be considered in cases of vitreous seeding from uveal melanoma.

Ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma in three consecutive cases.

PURPOSE: To describe the outcome of ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma. METHODS: Retrospective series of three consecutive patients with biopsy-proven choroidal lymphoma treated with ultra-low-dose radiotherapy. RESULTS: The three patients (two male, one female) of mean age 70 years (range, 64-74 years) demonstrated presenting visual acuity in the affected eye between 20/40 and 20/50. The choroidal lymphoma was unilateral in all cases and presented with multifocal yellow patchy choroidal infiltration, located in all four quadrants and measuring mean 2.9 mm (range, 1.9-4.0 mm) in thickness by ultrasonography. Anterior epibulbar extension of 5 mm diameter was noted in one case. By enhanced depth imaging optical coherence tomography, the choroidal infiltration demonstrated classic undulating appearance (n = 3), with subretinal fluid (n = 2) and intraretinal edema (n = 1). There was no systemic lymphoma in any case. Biopsy was performed in all three cases and was diagnostic (n = 1) or suggestive (n = 2) of B-cell lymphoma. Management involved ultra-low-dose radiotherapy (4 Gy delivered in two fractions, "boom-boom"). On follow-up (mean = 14 months, range = 6-24 months), complete tumor regression on ophthalmoscopy was documented in all three cases, with enhanced depth imaging optical coherence tomography and ultrasonography demonstrating evidence of lymphoma resolution and visual acuity improvement to 20/25-20/40. There were no radiation complications. CONCLUSION: In this small case series, ultra-low-dose (boom-boom) radiotherapy was effective for choroidal lymphoma with favorable response and minimal side effects.

VITREOUS AND PRERETINAL SEEDING AFTER TRANSVITREAL FINE NEEDLE ASPIRATION BIOPSY OF CHOROIDAL MELANOMA.

BACKGROUND/PURPOSE: To report vitreous and epiretinal seeding of choroidal melanoma after transvitreal fine-needle aspiration biopsy (FNAB). METHODS: A 74-year-old Caucasian man with a medium-sized, dome-shaped choroidal melanoma without Bruch membrane rupture, retinal invasion, or vitreous seeding. Transvitreal FNAB was performed for cytopathologic evaluation using a long 27-gauge needle attached to a 10-mL syringe by connector tubing. After passing through the pars plana, vitreous cavity, neurosensory retina, and Bruch membrane, the needle was inserted into the tumor and aspiration was performed. RESULTS: Cytopathologic evaluation revealed spindle B melanoma cells, and the tumor was subsequently treated with an 18-mm round Iodine-125 radioactive plaque delivering a tumor apex radiation dose of 7,000 cGy. Transscleral FNAB for cytogenetic evaluation performed at the time of plaque application disclosed chromosome three monosomy and chromosome six and eight disomy. Vitreous pigmented cells were first noted 14 months after brachytherapy. Funduscopy and optical coherence tomography at 24 months after plaque radiotherapy showed extensive vitreous and preretinal pigmented seeding that completely covered the optic disk. Fine-needle aspiration biopsy of the vitreous confirmed the presence of malignant melanoma cells, and the right eye was enucleated. Histopathologic evaluation showed a totally necrotic choroidal melanoma and an extensive malignant epiretinal membrane that covered and extended into the substance of the optic nerve head and focally invaded the neurosensory retina. The patient developed liver metastasis 3 years after the initial presentation and died 10 months later. CONCLUSION: This case demonstrates the rare development of extensive vitreous and preretinal seeding with retinal and optic nerve invasion after transvitreal FNAB of choroidal melanoma.

Intraocular schwannoma with extrascleral extension.

PURPOSE: To report a rare case of intraocular schwannoma with extrascleral extension in a patient with juvenile idiopathic arthritis and to review the literature for this topic. METHODS: Case report. RESULTS: A 19-year-old male with a history of juvenile idiopathic arthritis was referred for diagnosis and management of an episcleral mass, initially thought to be focal nodular scleritis. The ocular surface of the right eye revealed an elevated amelanotic episcleral nodule inferonasally, with thin strands of overlying sclera, feeding episcleral vessels, and measuring 11 mm × 11 mm in diameter and 5 mm in thickness. Ophthalmoscopic examination revealed a minimally pigmented ciliochoroidal tumor measuring 13 mm in diameter and 11.4 mm in total thickness and without associated subretinal fluid, orange pigment, or drusen. Ultrasound biomicroscopy and anterior-segment optical coherence tomography confirmed a solid mass with scleral disruption and extraocular extension. Shave biopsy revealed palisading spindle cells and interspersed eosinophilic fibrillary cytoplasmic processes, forming Verocay bodies. The specimen stained positive for S-100 and negative for Melan-A, consistent with benign schwannoma. Observation was recommended. CONCLUSION: Intraocular schwannoma is a rare, benign uveal tumor that can demonstrate extrascleral extension, mimicking inflammatory and malignant neoplastic processes. Clinical diagnosis is challenging, and tissue biopsy is required for definite diagnosis.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FEATURES OF VITREORETINAL LYMPHOMA IN 55 EYES.

PURPOSE: To evaluate spectral domain optical coherence tomography (SD-OCT) features of vitreoretinal lymphoma (VRL). METHODS: Review of records and SD-OCT images of vitreoretinal lymphoma evaluated at Ocular Oncology Service, Wills Eye Hospital between July 1, 2000, and April 1, 2019. RESULTS: There were 55 eyes of 32 patients included. At presentation, SD-OCT features included vitreous opacities (n = 36, 65%), preretinal deposits (n = 7, 13%), intraretinal deposits (n = 8, 15%), subretinal deposits (n = 20, 36%), retinal pigment epithelium abnormalities (n = 35, 64%), and subretinal pigment epithelium deposits (n = 35, 64%). Of 36 eyes with observed tumor progression, comparison (initial visit vs. time of progression) revealed more intraretinal deposits (17% vs. 50%, P = 0.005) at progression. Of 15 eyes with tumor recurrence, comparison (initial visit vs. time of recurrence) revealed more intraretinal deposits (7% vs. 47%, P = 0.04) at recurrence. At last visit, 39 eyes demonstrated tumor regression. By comparison (initial presentation vs. regression), there were less frequent vitreous opacities (67% vs. 0%, P < 0.001), intraretinal deposits (15% vs. 0%, P = 0.03), subretinal deposits (36% vs. 0%, P < 0.001), and subretinal pigment epithelium deposits (69% vs. 21%, P < 0.001) at regression. CONCLUSION: Using SD-OCT in patients with vitreoretinal lymphoma, local tumor regression correlated with a reduction in vitreous opacities, intraretinal deposits, subretinal deposits, and subretinal pigment epithelium deposits. SD-OCT is useful in judging vitreoretinal lymphoma response to therapy.

Photodynamic Therapy in Ocular Oncology.

Over the past two decades, we have witnessed the increasing use of photodynamic therapy (PDT) in the field of ocular oncology. Based on a review of the literature and our own experience, we herein review the role of PDT for the management of intraocular tumors. The discussion includes two main topics. First, we discuss the application of PDT for benign tumors, including circumscribed choroidal hemangioma, choroidal osteoma, retinal astrocytoma, retinal capillary hemangioma (retinal hemangioblastoma), and retinal vasoproliferative tumor. Second, we assess the role of PDT for malignant tumors, including choroidal melanoma and choroidal metastasis.

Unique Geospatial Accumulations of Uveal Melanoma.

PURPOSE: The main purpose of this paper was to describe the unique accumulation of cases of uveal melanoma (UM). All patients were white and did not have known occupational risk factors. From the authors' standpoint, there were no lifestyle factors in common in the reported cases. Results of more extensive analyses, including geospatial analysis, are currently being conducted and will be presented in a separate paper. DESIGN: Observational case series. METHODS: Descriptive data from medical records, patient interviews, and questionnaires were obtained from 5 patients from North Carolina, 6 patients from Alabama, and 14 patients from New York. Standard incidence ratio (SIR) calculations were provided by the respective states' cancer registries. UM is the most common primary malignant eye tumor in adults, although it is rare, with 2,500 cases diagnosed annually in the United States. Despite a growing understanding of the molecular characteristics, there remains uncertainty regarding epidemiologic trends and environmental risk factors. This study identified 3 geographic accumulations of UM: 1) Huntersville, NC; 2) Auburn, AL; and 3) Broome and Tioga Counties, New York. Investigation of these groups will guide ongoing efforts to discover potential risk factor and assist with future treatment and prevention. RESULTS: In North Carolina, 5 females who were identified as living in Huntersville, NC, were diagnosed with UM at ages 20, 22, 24, 30, and 31. The SIR calculations considering the observed and expected incidence ratios was 0.7 (95% confidence interval [CI], 0.5-0.9) in Mecklenburg County. In Alabama, 6 individuals who were identified as either attending Auburn University or employed there from 1989 to 1993 had diagnoses of UM. Initial SIR calculations for white females of all ages was 1.15 (95% CI, 0.989-1.328). In New York, SIR for Broome and Tioga counties were 0.93 and not significant. However, in Tioga county, for males and females and females alone, SIRs were 2.00 (P = .04) and 3.33 (P = .006). CONCLUSIONS: Although most of the conclusions that the SIR does not meet statistical criteria that defines these accumulations as true "cancer clusters," considering the incidence and demographics of UM, these accumulations of cases is unexpected and worth additional exploration. Further investigation into these cases with additional geospatial analyses and blood and tumor testing is ongoing. Information learned from the study of these unique populations may inform a better understanding of the pathogenesis of UM.