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Objective: This study aimed to develop a validated machine learning model to diagnose small choroidal melanoma. Design: This is a cohort study. Subjects, Participants, and/or Controls: The training data included 123 patients diagnosed as small choroidal melanocytic tumor (5.0–16.0 mm in largest basal diameter and 1.0 mm–2.5 mm in height; Collaborative Ocular Melanoma Study criteria). Those diagnosed as melanoma (n = 61) had either documented growth or pathologic confirmation. Sixty-two patients with stable lesions classified as choroidal nevus were used as negative controls. The external validation dataset included 240 patients managed at a different tertiary clinic, also with small choroidal melanocytic tumor, observed for malignant growth. Methods: In the training data, lasso logistic regression was used to select variables for inclusion in the final model for the association with melanoma versus choroidal nevus. Internal and external validation was performed to assess model performance. Main Outcome Measures: The main outcome measure is the predicted probability of small choroidal melanoma. Results: Distance to optic disc ≥3 mm and drusen were associated with decreased odds of melanoma, whereas male versus female sex, increased height, subretinal fluid, and orange pigment were associated with increased odds of choroidal melanoma. The area under the receiver operating characteristic “discrimination value” for this model was 0.880. The top four variables that were most frequently selected for inclusion in the model on internal validation, implying their importance as predictors of melanoma, were subretinal fluid, height, distance to optic disc, and orange pigment. When tested against the validation data, the prediction model could distinguish between choroidal nevus and melanoma with a high discrimination of 0.861. The final prediction model was converted into an online calculator to generate predicted probability of melanoma. Conclusions: To minimize diagnostic uncertainty, a machine learning-based diagnostic prediction calculator can be readily applied for decision-making and counseling patients with small choroidal melanoma.

Purpose: To identify non-overlapping diagnostic features between peripheral exudative hemorrhagic chorioretinopathy (PEHCR) and choroidal melanoma. Methods: A retrospective study of 80 patients (80 eyes); 40 patients (40 eyes) with PEHCR and 40 patients (40 eyes) with choroidal melanoma was conducted. Ophthalmoscopic and imaging features of PEHCR and choroidal melanoma were compared. Sensitivity and specificity for identifying PEHCR and choroidal melanoma were calculated. Youden’s J statistic was assessed for each diagnostic feature. Results: The most frequent clinical features of PEHCR were presence of druse (100%), hemorrhagic PED (93%), dome-shaped mass (B-scan) (90%), and subretinal/intraretinal hemorrhage (78%). Statistical analysis confirmed high sensitivity of hemorrhagic PED (0.93; 95% CI 0.80 – 0.98) and high specificity of clot retraction cleft, presence of lipid exudation and bilaterality (1.00; 95% CI 0.91 – 1.00) as diagnostic features of PEHCR. Statistical analysis revealed presence of subretinal fluid 0.80 (95% CI 0.54 – 0.91) was most sensitive and presence of orange pigment, mushroom shape on B-scan, ciliary body extension, and choroidal excavation were most specific (1.00; 95% CI 0.91 – 1.00) for choroidal melanoma. Non-overlapping diagnostic features of PEHCR were hemorrhagic PED, clot retraction cleft, presence of lipid exudation, and bilaterality. All PEHCR patients (100%) had at least one of these non-overlapping diagnostic features. Non-overlapping diagnostic features of choroidal melanoma were the presence of orange pigment, choroidal excavation, mushroom-shaped mass, and ciliary body extension (the latter 3 detected on B-scan). Youden’s J statistic was highest for hemorrhagic PED and lowest for dome shape appearance on B-scan (0.075). Conclusion: PEHCR and choroidal melanoma can be differentiated by identifying diagnostic features that are exclusive to each entity. The presence of hemorrhagic PED strongly supports a diagnosis of PEHCR. B-scan ultrasonography is required to detect a mushroom-shaped mass, choroidal excavation, or ciliary body extension to exclude underlying choroidal melanoma.

Uveal melanoma (UM) and renal cell carcinoma (RCC) can occur sporadically and as a manifestation of BAP1 tumor predisposition syndrome. We aimed to understand the prevalence of germ line BAP1 pathogenic variants in patients with UM and RCC. We reviewed patients managed at Cleveland Clinic between November 2003 and November 2019 who were diagnosed with UM and RCC. Charts were reviewed for demographic and cancer-related characteristics. RCC samples were tested for BAP1 protein expression using immunohistochemical (IHC) staining, and testing for germ line BAP1 pathogenic variants was performed as part of routine clinical care. Thirteen patients were included in the study. The average age at diagnosis of UM was 61.3 years. Seven patients underwent fine-needle aspiration biopsy for prognostic testing of UM (low risk =5, high risk =2). Twelve patients were treated with plaque radiation therapy, and 3 patients developed metastatic disease requiring systemic therapy. The median time to diagnosis of RCC from time of diagnosis of UM was 0 months. RCC samples were available for 7 patients for BAP1 IHC staining (intact =6, loss =1). All patients underwent nephrectomy (total = 3, partial = 8, unknown =2), and 1 received systemic therapy for metastatic RCC. Six patients underwent germ line BAP1 genetic testing. Of these, 1 patient was heterozygous for a pathogenic variant of BAP1 gene: c.1781-1782delGG, p.Gly594Valfs*48. The overall prevalence of germ line BAP1 pathogenic variants in our study was high (1/6; 17%; 95% CI 0–46%). Patients with UM and RCC should be referred for genetic counseling to discuss genetic testing.

Purpose: The aim of the study was to evaluate equivalence of growth rate and pathologic confirmation in small choroidal melanoma (SCM). Design: This study is a case series. Subjects, Participants, and Controls: A total of 61 patients with a choroidal melanocytic tumor of size 5.0–16.0 mm in the largest basal diameter and 1.0–2.5 mm in thickness were classified into the pathology-confirmed group (n = 19), growth-confirmed group (n = 30), and with combined observations (n = 12). Methods: Distribution of clinical variables (age, gender, laterality, tumor dimensions, tumor location, and presence of orange pigment, subretinal fluid, drusen, and retinal pigment epithelial [RPE] atrophy) between the groups was analyzed. Patient and disease characteristics were summarized as the median and interquartile range for continuous variables and the frequency and percentage for categorical variables. Comparisons were made using the Wilcoxon rank sum test for continuous variables and either Fisher’s exact test or the χ2 test for categorical variables with a p value threshold of 0.05 for statistical significance. Growth rate (change in basal dimension/12 months) diagnostic of SCM was quantified. Main Outcome Measures: The primary aim of this study was to test the hypothesis that “growth” was diagnostic of SCM with the secondary aim of quantifying the malignant “growth rate” (growth rate of SCM). Results: The clinical characteristics among all 3 groups were similar except more patients with symptoms (68 vs. 20 vs. 42%, p = 0.004) and juxtapapillary location (p = 0.03) were in the pathology group than in the growth-confirmed group. Those in the combined and growth-confirmed groups had more patients with drusen (11 vs. 60 vs. 50%, p = 0.003) and RPE atrophy (11 vs. 23 vs. 67%, p = 0.003), respectively, than in the pathology group. The median time to detect growth was 9 months (range 3–26 months). The mean growth rate in basal dimension was 1.8 mm/12 months (range, 0.0–7.4 mm; [95% CI: 1.32–2.28]). Conclusions and Relevance: Choroidal melanocytic lesions exhibiting a defined growth rate can be clinically diagnosed as SCM without a need for biopsy.

Purpose: The published data on ocular survival following intravenous chemotherapy of retinoblastoma (RB) seems to be skewed by evolving practice patterns induced by use of intravitreal chemotherapy (iVitc). We aimed to explore potential role of iVitc for vitreous seeding for patients treated with intravenous chemotherapy (IVC). Methods: A literature search was performed to identify cases of RB treated with primary IVC prior to advent of iVitc by various search engines (PubMed, Medline, and Google) from 1992 to 2018. Studies were excluded if number of cases were less than 40 or lacked data related to type of recurrence and its treatment. Rates and patterns of recurrence and its management were categorized. Results: Out of 15 studies identified, only 10 studies (797 eyes) met the inclusion criteria. The mean age at presentation was 15.3 months (range 0–192.8 months). Unilateral cases represented 25% of the cohort. The ocular survival rate with primary IVC was 63% (500/797 eyes). Of the 297 eyes (37%) that failed IVC therapy, additional 99 eyes could be salvaged with EBRT (599/797 eyes, 75%). Remaining 198 eyes were enucleated (198/797 eyes 25%). K-M survival analysis could not be done due lack of sufficient data. Recurrences that occurred (mean 12.2 months) after completion of primary IVC included relapse of retinal tumor (143 eyes [48%]), vitreous seeding (73 eyes [25%]), subretinal seeding (49 eyes [16%]), or any combination (103 eyes [35%]). Out of 73 eyes with vitreous seeding, additional 66 eyes (90%) would have been salvaged with iVitc, potentially improving ocular survival rates to 71% (500 + 66/797). Conclusions: Evolving practice patterns of RB treatment have unfavorably skewed published ocular survival rates following IVC. With incorporation of iVitc, the ocular survival rates with IVC can be potentially improved to be non-inferior to those achieved with intra-arterial chemotherapy.

Purpose: To report a case of local metastasis of conjunctival melanoma, which may occur via extravascular migratory metastasis (EVMM), and discuss its clinical relevance in conjunctival melanoma tumor staging and possible management implications. Methods: Retrospective chart review of a single clinical case with clinicopathologic correlation. Results: A 65-year-old male referred due to local recurrence of conjunctival melanoma at the caruncle was successfully treated after two excisional procedures with negative sentinel lymph node biopsies. Forty-eight months after initial presentation, the patient developed a nodular lesion representing local recurrence in the ipsilateral upper tarsal conjunctiva, distant from the primary tumor site. Histopathology showed nodules in the substantia propria in the absence of primary acquired melanosis. The tumor cells were found along the extravascular surface without intralymphatic or intravascular tumor cells consistent with local metastasis. One possible mechanism is angiotropic microsatellitosis leading to local EVMM. Additional neck CT imaging showed no lymphadenopathy. Conclusion: EVMM via angiotropic microsatellitosis is another possible mechanism of noncontiguous local recurrence of conjunctival melanoma. Angiotropic microsatellitosis may represent a high-risk finding possibly related to increased melanoma-related mortality.

Background: Retinoblastoma (RB) is a potentially heritable childhood cancer that is vision- and life-threatening. Assessing the risk of inheriting RB is important for structuring ophthalmic and genetic screening of family members. Purpose: To create a free online application that integrates phenotypic, genetic, and familial relationships with clinical best practice surveillance guidelines for families with RB. Methods: The risk of germline RB1 gene mutation was assessed for first- and second-degree relatives of a proband under variable clinical scenarios, integrating age, phenotype, relationship data, and genotype (germline RB1 mutation status: detected, undetected, not tested). Based on the assessed risk of a germline RB1 mutation, recommendations regarding further genetic testing as well as ophthalmic surveillance were derived from consensus guidelines. Results: The recommendations depend on the RB1 germline mutation status (detected, undetected, not tested), which were further subcategorized by the results of tumor phenotype, relationship to proband, age of the relative, and family structure. The online application is available at https://nakul-singh.shinyapps.io/RB_Screening_rec/. Conclusions: The assessed risk of germline RB1 mutation determines ophthalmic surveillance recommendations. The tool may have most value in regions where access to specialized care is limited.

Purpose: Histologic correlation of clinical patterns of recurrent choroidal melanoma following I-125 plaque brachytherapy was performed to identify pathologic mechanisms of recurrence. Methods: We reviewed 7 cases of recurrent choroidal melanoma following I-125 plaque brachytherapy managed with enucleation. Clinical characteristics included tumor dimensions, radiation dose, time to local recurrence, and clinical pattern of recurrence. Histopathology (hematoxylin and eosin and periodic acid – Schiff) and immunohistochemistry (Ki-67, CD-163, HMB45, and SOX10) were performed. Results: Mean follow-up time and time to local recurrence were 42 and 21 months after brachytherapy, respectively. Tumor recurrences were described clinically as marginal in 43%, diffuse in 29%, and extraocular extension (EOE) in 29%. Eighty-six percent were classified as mixed cell type and 14% were epithelioid type. Tumor zonation (histologic demarcation between zones of recurrent and nonrecurrent tumor cells by immunohistochemistry) was present in marginal and EOE cases (n = 6) and absent in the diffuse cases (n = 2). Ki-67 proliferative index was higher in marginal and EOE recurrences, while diffuse cases showed uniform ­Ki-67 staining. CD-163 staining was found to be greater in nonrecurrent tumor. HMB45 correlated with SOX10 with a greater staining in recurrent tumor. Conclusion: Our observations provide a correlation between histopathologic and clinical patterns of local recurrence of choroidal melanoma after brachytherapy.

Objective: To describe treatment trends and outcomes of liver metastasis from uveal melanoma. Methods: Retrospective case series of 73 patients with uveal melanoma liver metastasis. Patients were treated first-line with systemic therapy (not including checkpoint inhibitors), checkpoint inhibitors, local therapy or no treatment. Time to metastasis, detection method, and survival data were collected. Time periods were divided between 2004–2011 and 2012–2016. Cox proportional hazards models were used to compare progression-free survival (PFS) and overall survival (OS). Results: Median PFS and OS for the entire cohort was 4 months (95% CI 3–5) and 15 months (95% CI 11–18), respectively. There was no statistically significant difference in PFS and OS across the two time periods. Patients who received no treatment had the shortest OS (median 4.9 months), whereas those treated with local therapy had the longest PFS (median 4.6 months) and OS (median 18.7 months). Having liver metastasis diagnosed by symptoms was associated with a greater risk of mortality (p < 0.001). Conclusion: Patients who received first-line local treatment had the longest PFS and OS, while patients who received no treatment had the shortest OS. Survival outcomes did not improve for patients including those receiving check point inhibitors.

Importance: Diagnosis of small choroidal melanoma is based upon clinical features and presence of factors predictive of local malignant growth. Prognostic biopsy quantifies risk of metastasis. Objective: The aim of this study is to explore relationship between clinical characteristics and metastatic potential of a small choroidal melanoma. Design: Retrospective review of 53 patients with small choroidal melanoma treated in a tertiary oncology clinic. Patients were derived from 3 cohorts, with pathologic confirmation, with growth confirmation, and those treated only on clinical basis. Based upon prognostic biopsy outcomes, each case was classified into low or high metastatic potential groups. Distribution of clinical characteristics such as age, laterality, symptoms, tumor dimensions, tumor distance from optic nerve and fovea, presence of surface orange pigment, drusen, retinal pigment epithelial atrophy, and subretinal fluid was analyzed between metastatic groups. Main Outcome Measures: Distribution of clinical characteristics between low or high metastatic potential groups was analyzed. Results: A total of 53 patients [mean age, 61 years (range, 27–81 years); 32 (60%) men and 21 (40%) women] were classified into pathology confirmed group (n = 13), growth confirmed group (n = 26), and with clinical group (n = 14). Prognostic biopsy in the growth, pathology, and clinical groups revealed low metastatic potential in 23, 10, and 11 patients, respectively, and high metastatic potential in 3 patients in each group. Distribution of clinical characteristics between low or high metastatic potential groups was not statistically significantly different. Conclusion: Clinical characteristics do not identify metastatic potential of a small choroidal melanoma.

Uveal melanoma (UM) and renal cell carcinoma (RCC) can occur sporadically and as a manifestation of BAP1 tumor predisposition syndrome. We aimed to understand the prevalence of germ line BAP1 pathogenic variants in patients with UM and RCC. We reviewed patients managed at Cleveland Clinic between November 2003 and November 2019 who were diagnosed with UM and RCC. Charts were reviewed for demographic and cancer-related characteristics. RCC samples were tested for BAP1 protein expression using immunohistochemical (IHC) staining, and testing for germ line BAP1 pathogenic variants was performed as part of routine clinical care. Thirteen patients were included in the study. The average age at diagnosis of UM was 61.3 years. Seven patients underwent fine-needle aspiration biopsy for prognostic testing of UM (low risk =5, high risk =2). Twelve patients were treated with plaque radiation therapy, and 3 patients developed metastatic disease requiring systemic therapy. The median time to diagnosis of RCC from time of diagnosis of UM was 0 months. RCC samples were available for 7 patients for BAP1 IHC staining (intact =6, loss =1). All patients underwent nephrectomy (total = 3, partial = 8, unknown =2), and 1 received systemic therapy for metastatic RCC. Six patients underwent germ line BAP1 genetic testing. Of these, 1 patient was heterozygous for a pathogenic variant of BAP1 gene: c.1781-1782delGG, p.Gly594Valfs*48. The overall prevalence of germ line BAP1 pathogenic variants in our study was high (1/6; 17%; 95% CI 0–46%). Patients with UM and RCC should be referred for genetic counseling to discuss genetic testing.

Background: The liver is the most common site for metastases of several primary malignancies including uveal melanoma. Methods: Review of imaging characteristics of incidental common benign liver lesions including hepatic cyst, hemangioma, focal nodular hyperplasia, and hepatic adenoma and contrasting them with uveal melanoma metastases. Results: Benign hepatic lesions may be cystic or, if solid, relatively stable in size over time. For hepatic lesions larger than 10 mm in size, characteristic imaging features typically allow for confident diagnosis. When lesions are small (less than 10 mm), definitive characterization can be difficult. Moreover, lesions smaller than 10 mm can be difficult to biopsy under ultrasound or computed tomography (CT) guidance, and short-term follow-up will often be useful to assess for stability or progression. Overall, magnetic resonance imaging is more specific than CT scan and at least as sensitive as CT for detecting uveal melanoma liver metastases. Conclusions: New multiple enhancing solid liver lesions should raise suspicion of uveal melanoma liver metastases. Discussion of challenging cases with the radiologist may be beneficial, as pertinent information such as size, location, and molecular prognostication status of the primary tumor can guide radiological interpretation of hepatic lesions.

Purpose: To analyze trends in incidence, treatment, and survival of uveal melanoma in the United States from 1973 to 2013 using the Surveillance, Epidemiology, and End Results database. Materials and Methods: Patients were identified using International Classification of Disease for Oncology codes: C69.3 (choroid), C69.4 (ciliary body and iris), and C69.2 (retina). Trends in age-adjusted incidence, treatment (surgery or radiation), and 5-year relative survival were calculated. Results: There were 4,999 cases of uveal melanoma. The majority (97.8%) were reported by hospital inpatient/outpatient clinics. Histopathologic confirmation was available in 67.8%. The mean age-adjusted incidence was 5.2 per million (95% CI 5.0-5.4). When the incidence was standardized for race, a small but statistically significant (p < 0.05) annual percentage change of 0.5% was detected in Whites. There was a decline in patients treated with surgery alone (94.2% from 1973 to 1975 vs. 24.7% from 2012 to 2013). A corresponding increase was observed in radiation as primary treatment selection (1.3% from 1973 to 1975 vs. 68.3% from 2012 to 2013). No change in the 5-year relative survival (80.9%) was observed. Conclusions: The age-adjusted incidence of uveal melanoma has remained stable with a minor increase in Whites. Despite a shift towards globe-preserving treatment, there has not been a concomitant improvement in survival.

Purpose: To report 3 cases providing insight into clinical progression of reactive retinal astrocytic tumor. Methods: The clinical, imaging, and when available, the cytologic features of 3 cases of reactive retinal astrocytic tumor (focal nodular gliosis) were reviewed. Results: A 6-year-old female, a 49-year-old man, and a 39-year-old man each developed a white retinal mass associated with laser photocoagulation, lattice degeneration, and treatment of a presumed vascular tumor, respectively. All tumors were white, circumscribed retinal masses that tended to be associated with exudation and either initially or eventually minimal vascularity. Conclusion: Reactive retinal astrocytic tumor can be observed in response to a degenerative, inflammatory, or ischemic retinal insult. Such tumors may progress after therapeutic intervention.

Purpose: To report a case of dural arteriovenous fistula presenting with purely contralateral ophthalmic manifestations. Design: Interventional case report. Results: A 70-year-old male presenting with right-sided proptosis, injection, and choroidal effusions with a completely quiet fellow eye was found to have a left-sided dural arteriovenous fistula. After recognition on cerebral angiography, successful endovascular embolization was achieved, along with complete resolution of the contralateral ocular signs. Conclusions: Entirely unilateral ocular signs suggestive of an intracranial arteriovenous fistula may herald the presence of a contralateral vascular lesion.

Aim: The objective was to evaluate the relationship between the regression rate of ciliary body melanoma and choroidal melanoma after brachytherapy and chromosome 3 monosomy status. Methods: We conducted a prospective and consecutive case series of patients who underwent biopsy and brachytherapy for ciliary/choroidal melanoma. Tumor biopsy performed at the time of radiation plaque placement was analyzed with fluorescence in situ hybridization to determine the percentage of tumor cells with chromosome 3 monosomy. The regression rate was calculated as the percent change in tumor height at months 3, 6, and 12. The relationship between regression rate and tumor location, initial tumor height, and chromosome 3 monosomy (percentage) was assessed by univariate linear regression (R version 3.1.0). Results: Of the 75 patients included in the study, 8 had ciliary body melanoma, and 67 were choroidal melanomas. The mean tumor height at the time of diagnosis was 5.2 mm (range: 1.90-13.00). The percentage composition of chromosome 3 monosomy ranged from 0-20% (n = 35) to 81-100% (n = 40). The regression of tumor height at months 3, 6, and 12 did not statistically correlate with tumor location (ciliary or choroidal), initial tumor height, or chromosome 3 monosomy (percentage). Conclusion: The regression rate of choroidal melanoma following brachytherapy did not correlate with chromosome 3 monosomy status.

Background: To expand the spectrum of ophthalmic manifestations in cat scratch disease. Methods: Case report. Results: A 7-year-old male was referred for evaluation of his left optic disc after failing vision screening test at school. His visual acuity was 20/20 OD and light perception OS. Fundus examination showed a left optic disc lesion associated with an exudative retinal detachment and vitreous seeding. Ultrasonography revealed a 7 × 7.5 × 3.8 mm lesion with a possible 6.3 mm of retrolaminar extension into the substance of the optic nerve. Brain MRI did not show evidence of optic nerve involvement but revealed a 6-mm nodule of the pineal gland suggestive of a pineoblastoma. Enucleation was performed and histopathology revealed a suppurative granulomatous inflammation suggestive of Bartonella infection. Upon further questioning, the patient had recent exposure to kittens with areas of cat scratches along both of his arms. He was subsequently referred to and treated with a 2-week course of trimethoprim-sulfamethoxazole and rifampin by the pediatric infectious disease specialist. Repeat brain MRI showed interval total resolution of enlarged pineal gland. Conclusion: Optic nerve granulomas are a rare presentation of cat scratch disease and could potentially masquerade as retinoblastoma.

Aim: To develop an automated algorithm to quantify ultrasonographic A-scan parameters of choroidal melanoma. Methods: The study included 100 consecutive patients with a clinical diagnosis of choroidal melanoma. Ultrasonographic A-scans (8 MHz, 1,550 m/s, tissue sensitivity = 67 dB) were performed by standard techniques. We created and then utilized a MATLAB® script to generate four quantifiable A-scan parameters: (1) tumor height (mm), (2) the number of internal reflectivity peaks (numerical value), (3) median internal reflectivity (%), and (4) angle κ (°). Results: There were small (≤2.5 mm, n = 32), medium (2.6–10.0 mm, n = 53), and large (> 10.0 mm, n = 14) tumors. The mean number of internal reflectivity peaks counted between the two tumor boundary spikes (surface and base) was 10.0 (σ = 8.7, range 1–37). The median value of the internal reflectivity peaks for all cases varied from 19.8 to 99.5 (mean = 68.3, σ = 20.5). A statistically significant correlation was observed between the tumor height categories and each of the three A-scan parameters: the number of internal reflectivity peaks (ρ = 0.90, p < 0.01), median internal reflectivity (ρ = –0.63, p < 0.01), and a positive angle κ (ρ = –0.32, p = 0.03). Conclusions: An automated algorithm can provide quantifiable A-scan parameters for choroidal melanoma.

Purpose: To discuss the diagnostic and management challenges of a ciliary body tumor in an African-American patient. Procedures: Our 52-year-old African-American female patient, referred with a nasal ciliary body tumor in the right eye, underwent investigations including A- and B-scan ultrasonography, Optos fundus photography and fundus fluorescein angiography at clinical consultation. A diagnostic fine-needle aspiration cytology and open flap biopsy were undertaken. Results: Histopathology and cytology confirmed a malignant melanoma, which is extremely rare in the African-American population. The patient was treated with brachytherapy with an iodine-131 plaque ensuring adequate coverage of the tumor site but, at the same time, taking special precaution not to disturb the biopsy site. Conclusions: The diagnosis of a ciliary body tumor, especially in an African-American patient, can be challenging despite advances in imaging systems, given the multitude of differential diagnoses. Uveal melanomas are extremely rare in African-Americans. On detailed questioning, our patient reported having mixed ancestry, with Caucasian ancestors on the mother's side and native Indian ancestors on the father's side. Information on true ethnicity should be specifically asked for as it alters the incidence rate. Biopsy provides the only option for a correct diagnosis and for developing a management plan. Fine-needle aspiration biopsy can be diagnostic, though an open flap biopsy is superior as it provides information on tissue architecture and the possibility of immunohistochemistry. Also, with plaque brachytherapy treatment, special care needs to be taken not to disturb the biopsy site to reduce the risk of seeding, and an innovative approach may need to be taken to ensure adequate radiation coverage of these anterior tumors.

Conjunctival melanoma (CoM) is a rare and aggressive form of melanoma. There is a lack of consensus on a unified management plan for this disease. Recently, a few centers have adopted the regional sentinel lymph node biopsy into the staging process of CoM. This study presents a critical assessment of the role of sentinel lymph node biopsy in CoM and presents a simplified management algorithm based on high-risk clinical and pathological features.

A 50-year-old female who had undergone enucleation in the left eye and brachytherapy in the right eye for retinoblastoma at the age of 2 years was diagnosed with nonproliferation radiation retinopathy 47 years following the initial treatment. The patient had noticed black spots in her vision (scotomas) that interfered with reading. New onset of microaneurysms and lipid exudation threatening the foveola was noted on examination of the right eye. Initial visual acuity (VA) was 20/25. Optical coherence tomography showed no evidence of macular edema, but parafoveal lipid exudation was present. On fluorescein angiography, no sign of neovascularization or macular ischemia was observed. Direct focal treatment of microaneurysms was performed to prevent progression of the radiation retinopathy and vision loss. At 18 months' follow-up following focal laser, VA remained stable at 20/25 and there was a regression of the retinopathy.

The survival of patients with uveal melanoma remains poor because of the development of metastatic disease. Adjuvant therapy after treatment of the primary tumor has been tested but has not been shown to prevent the development of metastasis. Several new approaches are being developed. Cytotoxic and immunotherapeutic regimens are being more rationally applied using tumor genetic criteria to better identify patients at risk. Trials in the adjuvant setting of novel immunotherapeutic and targeted agents active in the metastatic setting are being developed, as are approaches to promote cellular differentiation and dormancy. The rarity and biology of uveal melanoma present challenges. Participation in well-designed, scientifically sound clinical trials is critical.