Objective: To determine the prevalence of retinal astrocytic tumors in newborns. Methods: Analysis of published articles describing large-scale screening studies of newborns (pre-term and term) performed using wide-field digital fundus imaging in which authors reported fundus lesions and abnormalities other than retinopathy of prematurity and retinal hemorrhages; counting of the cases in which an ocular fundus lesion diagnosed as retinal astrocytoma or retinal astrocytic hamartoma was reported. Results: Fourteen published articles on ocular fundus screening examinations of > 1,000 newborns using digital wide-field fundus imaging (13 articles) or smart phone fundus imaging (1 article) reported fundus lesions and abnormalities other than retinopathy of prematurity in an aggregate total of over 300,000 cases. Only 1 newborn was reported to have a discrete retinal tumor diagnosed as a retinal astrocytic tumor by the authors. Conclusion: Although a few congenital lesions diagnosed as retinal astrocytic tumors have been documented, most lesions of this type do not appear to be congenital. In view of this, the term “retinal astrocytic hamartoma” (implying a congenital malformation) for all lesions of this type appears to be inappropriate.

BACKGROUND AND OBJECTIVE:Retinal gliovascular proliferation (RGVP) is a benign lesion of the retina that can arise idiopathically or secondary to another disease entity. This study describes the clinical features, treatment, and outcomes of six patients with secondary RGVP associated with irradiated, regressed retinoblastoma, and distinguishes it from late local relapse of retinoblastoma. PATIENTS AND METHODS:In a retrospective review of available clinical records of 550 patients evaluated for retinoblastoma in a single ocular oncology practice between 1975 and 2022, seven eyes of six patients were identified as having secondary RGVP overlying a treated and regressed retinoblastoma. The clinical features, treatment, and outcomes are described. RESULTS:The median age at RGVP diagnosis was 20 years. All RGVPs were associated with a completely regressed retinoblastoma and in proximity to a calcific tumor residue or chorioretinal atrophy that remained after external beam radiotherapy (six eyes) or plaque brachytherapy (one eye). Lesions were measured between 2.8 to 12 mm in largest basal diameter and 1.3 to 4.4 mm in thickness and described as globular, raised areas with focal retinal telangiectasis often associated with overlying subretinal fluid or hemorrhage. Median time between initial retinoblastoma treatment and detection of RGVP was 20 years. Treatment was decided based on evidence of lesion growth and most often consisted of laser photocoagulation and intravitreal anti-VEGF injection. Through available follow-up of the treated lesions, all exhibited at least partial regression, while two untreated lesions remained stable, reassuring us against late local relapse of retinoblastoma. CONCLUSIONS:Secondary RGVP develops occasionally in association with regressed previously irradiated retinoblastoma. This lesion must be distinguished from late local relapse of active retinoblastoma. [Ophthalmic Surg Lasers Imaging Retina 2024;55:714-720.].

Background: To describe the clinical features and course of a series of survivors of retinoblastoma who developed a non-pineoblastoma second primary malignant neoplasm (SPMN). Methods: A retrospective review of patients treated for retinoblastoma who developed a SPMN was performed. The demographics, clinical features and treatments for retinoblastoma, pathologic types of SPMN, the intervals between the retinoblastoma diagnosis and treatment and diagnosis of non-pineoblastoma SPMN, treatment provided for the SPMN, and the survival outcomes of the patients were evaluated. Results: Of 550 patients treated initially for retinoblastoma, this series used the 15 (2.7%) that developed a non-pineoblastoma SPMN, 14 of which (93.3%) had been treated for bilateral retinoblastoma. All patients in this series had germline retinoblastoma. The median time from retinoblastoma diagnosis to SPMN diagnosis was 19.0 years (extremes 3.4 and 39.4 years). Six of the fifteen patients died during the follow-up of their SPMN. Nine patients were still alive without active residual SPMN at the last follow-up. The median interval between initial retinoblastoma diagnosis and death in the 6 patients who died of their SPMN was 18.8 years (extremes 6.2 and 34.6 years) and between diagnosis of the SPMN and death was 1.2 years (extremes 0.25 and 4 years). Conclusion: A SPMN occurred in this series in 2.7% of retinoblastoma survivors, and all occurred in patients with germline retinoblastoma. All patients with SPMN who had been treated by EBRT developed the SPMN in the field of prior radiation.

Midzonal iris pigment epithelial (IPE) cysts are typically asymptomatic but can pose a differential diagnostic challenge by mimicking ciliary body melanoma. We conducted a case series study of the clinical characteristics of these iris cysts, particularly their shapes and sizes and associated lesions revealed by ultrasound biomicroscopy (UBM). Charts of patients with midzonal IPE cysts encountered by one or more of the authors were reviewed retrospectively. Pertinent demographic information and clinical data about these patients and their cysts were abstracted. 69 patients were identified. At initial diagnosis, patients ranged in age from 14.8 to 89.5 years (median: 66.0 years). Only one patient was < 30 years old at diagnosis. 38 patients (55.1 What is new

Isolated choroidal melanocytosis is a rare condition that appears to be a limited form of ocular melanocytosis. Ocular melanocytosis has been known to be associated with an increased risk of uveal melanoma, and more recently, a similar association has been suggested for isolated choroidal melanocytosis. We describe 3 cases of patients who developed unilateral, multifocal uveal melanoma in the setting of underlying isolated choroidal melanocytosis. All patients developed either two distinct tumors at presentation or a new discrete choroidal melanoma arising from the choroidal melanocytosis over 1 year following treatment of the original tumor by plaque brachytherapy. These cases provide additional evidence of the association between isolated choroidal melanocytosis and uveal melanoma and suggest increased risk of multifocal melanoma in patients with this condition.

The present research investigates the pharmacokinetics and toxicity of a chitosan (CS) and poly(lactic-co-glycolic) acid (PLGA)-based methotrexate (MTX) intravitreal micro-implant in normal rabbit eyes. PLGA and CS-based micro-implants containing 400 µg of MTX were surgically inserted in the vitreous of twenty-four New Zealand rabbits using minimally invasive procedures. The PLGA-coated CS-MTX micro-implant and the placebo micro-implant were inserted in the right eye and in the left eye, respectively, of each rabbit. The intravitreal MTX concentration was evaluated on Days 1, 3, 7, 14, 28 and 56. A therapeutic concentration of MTX (0.1–1.0 µM) in the rabbit vitreous was observed for 56 days. The release of MTX in the therapeutic release phase followed first-order kinetics. Histopathologic evaluation on Days 14, 28 and 56 of the enucleated eyes demonstrated no signs of toxicity or any anatomical irregularity in the vitreoretinal domain. Additionally, the micro-implants were stationary at the position of their implantation throughout the duration of the study. The PLGA-coated CS-MTX micro-implant can serve as a potential alternative to the current treatment modality of intravitreal MTX injections based on its performance, thereby avoiding associated complications and the treatment burden of multiple injections.

Focal aggregate of normal or near normal uveal melanocytes (FANNUM) of the choroid is a term the author has proposed to categorize small melanocytic choroidal lesions that are not detectably thicker than surrounding normal choroid by B-scan ocular ultrasonography. In this article, the author describes the clinical features of small melanotic choroidal lesions he categorizes clinically as FANNUMs and discusses the presumed compositional spectrum of such lesions. He explains why such lesions should not be categorized uniformly as small choroidal nevi. Lesions of this type are detectable in at least one eye of approximately 25% of lightskinned blue-eyed persons over the age of 50 years. They are frequently multifocal in the affected eye, bilateral, or both.

Purpose: To evaluate the safety and toxicity profile of a chitosan (CS) and poly(lactic-co-glycolic) acid (PLGA)based sustained release methotrexate (MTX) intravitreal micro-implant in normal rabbit eyes using non-invasive testing that included electroretinography (ERG), ultrasound biomicroscopy (US), slit-lamp biomicroscopy (SLB), funduscopy, and intraocular pressure (IOP). Methods: PLGA-coated CS-based micro-implants containing 400 ?g of MTX and placebo (without drug) microimplants were surgically-implanted in the vitreous of the right and the left eyes, respectively, in each of the thirty New Zealand rabbits. ERG, US, SLB, funduscopy, and IOP were assessed in both eyes at pre-determined time points (days: 1, 3, 7, 14, 28 and 56). The safety of micro-implants was assessed by analyzing the ERG data using different statistical models, to quantify and compare the functional integrity of the retina. Further, US, funduscopy, SLB and IOP determined the condition of the retina, the micro-implant and associated intraocular features. Results: Statistical analyses of the ERG data showed unchanged functional integrity of retina between eyes with the PLGA-coated CS-based MTX micro-implant and the placebo micro-implant. US analysis showed that microimplants were stationary throughout the study. SLB, funduscopy and IOP further confirmed that there were no abnormalities in the intraocular physiology. Conclusion: The findings from ERG, US, SLB, funduscopy, and IOP showed no detectable adverse effects caused by our biodegradable micro-implants. These non-invasive techniques appeared to show lack of significant ocular toxicity over time in spite of degradation and changes in morphology of the micro-implants following intraocular implantation.

Purpose Isolated choroidal melanocytosis is a congenital melanocytic hyperpigmentation involving the choroid that is not associated with iridic or scleral features of ocular melanocytosis. The purpose of this work was to describe the clinical features and course of a relatively large series of patients with this disorder. Methods A retrospective clinical study of 37 patients with isolated choroidal melanocytosis encountered in a single practice 1986–2018 was done. All lesions were 5 mm or larger in the largest basal diameter, homogeneously melanotic, and completely flat by conventional ocular ultrasonography. Results The 37 patients ranged in age from 2 weeks to 87 years (mean 31.5 years, median 18 years) at initial diagnosis of the melanotic choroidal lesion. Arc length largest basal diameter of the melanotic choroidal lesion ranged from 5.5 to 37 mm (mean 14.6 mm, median 13 mm). The lesion extended beneath the fovea in 18 eyes and to the optic disc margin in 6 eyes. Ten of the lesions straddled the ocular equator, but the center point of all of the lesions was posterior to the equator. The retina was fully attached and appeared normal over the melanotic choroidal lesion in each of these eyes. None of the melanotic choroidal lesions exhibited clumps of orange pigment or drusen on its surface. The lesion was unilateral and unifocal in 36 of the 37 patients. One patient had bilateral choroidal melanocytosis that was isolated in one eye but associated with partial iris melanocytosis in the fellow eye. Three adult patients had a choroidal melanoma localized to the patch of choroidal melanocytosis at baseline. One other adult patient had a choroidal melanoma in the fellow eye at baseline. One pediatric patient had viable unilateral non-familial retinoblastoma in the fellow eye and two adult patients had a classic choroidal nevus in the fellow eye. None of the flat patches of choroidal melanocytosis that were monitored periodically after initial diagnosis expanded appreciably during follow-up ranging from 4.9 months to 15.2 years (mean 5.0 years, median 2.3 years). Conclusions Isolated choroidal melanocytosis is a distinct clinical entity that must be distinguished from broad-based choroidal nevus, choroidal melanocytoma, small choroidal malignant melanoma, acquired bilateral patchy-streaky choroidal melanocytic fundopathy associated with disorders such as cutaneous vitiligo and Waardenburg syndrome, acquired bilateral zonal choroidal melanocytic fundopathy, and diffuse uveal melanocytic proliferation associated with systemic cancer. This disorder appears to predispose affected eyes to development of choroidal melanoma arising from the hypermelanotic patch.

Purpose To define, describe, and illustrate a previously unreported category of discrete melanotic choroidal melanocytic lesion. Methods Prospective ophthalmoscopic study of the ocular fundi of 79 light-skinned persons 50 years of age or older not referred for any evident fundus lesion, with detection of all evident discrete melanotic choroidal lesions > 0.3 mm in largest basal diameter. Results One or more discrete dark-brown to gray choroidal lesions > 0.3 mm in largest basal diameter were detected in 27 of the 79 evaluated subjects (34.2%). All but four of the detected lesions were “flat” by both ophthalmoscopy and ultrasonography. A single flat lesion was present in one eye of 14 subjects whose fellow eye was normal, 2 or more flat lesions were evident in one eye of 5 subjects whose other eye was normal, and one or more lesions were evident in both eyes of 6 subjects. Conclusion While some of the discrete small, flat melanocytic choroidal lesions detected in this study might have been choroidal nevi, the author hypothesizes that an indeterminate proportion of them may have been focal aggregates of normal or near normal uveal melanocytes (FANNUMs).

We report the case of a 9-month-old girl with bilateral retinoblastoma who had incomplete tumor resolution after selective ophthalmic artery infusion chemotherapy (SOAIC). Systemic chemotherapy, rarely used as salvage therapy after SOAIC, with systemic carboplatin, etoposide, and vincristine achieved complete and sustained regression in both eyes.

Purpose: To study the feasibility of using Cherenkov luminescence imaging (CLI) toevaluate and document ruthenium plaque position during episcleral brachytherapy for choroidal melanoma.Methods: Ruthenium-106 decays to rhodium-106 by emitting high-energy beta particles. When the electrons propagate through the eyewall, so-called Cherenkov radiation generates visible light, which can be captured by high-sensitive cameras. Five consecutive patients with uveal melanoma located in the posterior pole were studied. All patients underwent brachytherapy with a Ru-106 plaque. The tumors were 6.0-10.2 mm in largest basal diameter and 1.93.5 mm in thickness. The plaques had an activity between 7.7-19.1 MBq at the time of examination (24-48 hours after implantation). CLI was performed in complete darkness by a cooled EMCCD-camera (Andor iXon DV887) mounted on a fundus camera (Kowa RC-XV2) modified for long exposures.Results: CLI revealed the actual plaque position by displaying a circular spot of light in the fundus corresponding to the plaque area. The Cherenkov light appeared as a halo surrounding the tumor, which showed some asymmetry if the plaque was slightly displaced. There was a positive correlation between plaque activity and light intensity, and exposure times between 30-60 seconds were necessary to achieve the desired image quality. However, the long exposures made it difficult to maintain stable eye fixation and optimal image sharpness.Conclusion: CLI is a feasible method to assess and document radioactive plaque position in brachytherapy of uveal melanoma. Its main limitation is the long exposure time, which may be solved by improving camera sensitivity and eye fixation. Presentation Title: The McCannel Brachytherapy Plaque Quick Release Stitch Authors: Colin McCannel, Tara McCannel Stein Eye Institute/UCLA, Los Angeles, USA Abstract: Purpose:To describe a technique to suture brachytherapy plaques in place that allows simplified, efficient removal.Methods:Brachytherapy plaques can be secured to the sclera by means of a quick removal suturing technique. 5-0 VicrylTMsuture is passed through the sclera. A loop of the VicrylTMsuture is threaded through the islets of the flange of the brachytherapy plaque. A 2-0 Prolene® suture is passed through the VicrylTMloops above the flange, followed by tightening and tying of the VicrylTMsuture. For release, the 2-0 Prolene® suture is pulled out and the plaque can then be pulled out by means of a “handle suture” previously attached to the flange.Results:The technique works as expected allowing securing the plaque to the sclera firmly, but allows removal without having to find and cut the sutures that hold the plaque in place. This technique has been used in 187 cases without complications related to the technique of securing the plaque. Among the cases in which the McCannel plaque stitch was used, there have not been any recurrences with a mean follow-up 0f 18 months.Conclusions:The McCannel plaque stich can be used to secure and later efficiently remove brachytherapy plaques. Currently there is no detectable disadvantage. Presentation Title: Regression Patterns of Choroidal Melanoma after Plaque Brachytherapy Abhilasha Maheshwari1,2, Paul Finger2 1Centre for Sight, Hyderabad, India. 2New York eye cancer center, New York, USA Abstract: Purpose: To describe the patterns of regression of choroidal melanoma (CM) after treatment with plaque brachytherapy.Methods: Retrospective interventional case series including 170 consecutive patients treated with 103Pd plaque radiation for CM. Outcome measures were changes in tumor thickness, surface characteristics, tumor vascularity, ultrasonography, fluorescein angiography, optical coherence tomography and histopathology.Results: The mean initial tumor thickness of 3.9-mm decreased to 1.7-mm after plaque brachytherapy. On imaging, tumors were pigmented in 51%(n=86/170), amelanotic in 10%(n=17/170) and variably pigmented in 39%(n=67/170). Tumor pigmentation increased in 64%(n=106/166), decreased in 18%(n=30/166) and was unchanged in 18%(n=30/166). Of the 120 that demonstrated intrinsic vascularity, 90%(n=108/120) showed complete resolution. Subretinal fluid (SRF) was present in 34%(n=58/170) at presentation, of which, 15%(n=9/58) had persistent SRF at last follow-up. On ultrasound imaging, 88%(n=149/170) tumors presented with low-moderate internal reflectivity of which 61%(n=91/149) showed increased reflectivity on regression. We noted a crescendo-decrescendo fluctuation of orange pigment lipofuscin (OP) along with complete resolution of drusenoid Purpose: To describe the patterns of regression of choroidal melanoma (CM) after treatment with plaque brachytherapy.Methods: Retrospective interventional case series including 170 consecutive patients treated with 103Pd plaque radiation for CM. Outcome measures were changes in tumor thickness, surface characteristics, tumor vascularity, ultrasonography, fluorescein angiography, optical coherence tomography and histopathology.Results: The mean initial tumor thickness of 3.9-mm decreased to 1.7-mm after plaque brachytherapy. On imaging, tumors were pigmented in 51%(n=86/170), amelanotic in 10%(n=17/170) and variably pigmented in 39%(n=67/170). Tumor pigmentation increased in 64%(n=106/166), decreased in 18%(n=30/166) and was unchanged in 18%(n=30/166). Of the 120 that demonstrated intrinsic vascularity, 90%(n=108/120) showed complete resolution. Subretinal fluid (SRF) was present in 34%(n=58/170) at presentation, of which, 15%(n=9/58) had persistent SRF at last follow-up. On ultrasound imaging, 88%(n=149/170) tumors presented with low-moderate internal reflectivity of which 61%(n=91/149) showed increased reflectivity on regression. We noted a crescendo-decrescendo fluctuation of orange pigment lipofuscin (OP) along with complete resolution of drusenoid retinal pigment epithelial detachments (DRPED). In the entire series of 170 patients, there was 0.5%(1) failure of local control, 2%(4) secondary enucleations and 6%(10) patients developing metastasis.Conclusion: Findings related to choroidal melanoma regression after 103Pd plaque brachytherapy included decreased intrinsic tumor vascularity, decreased tumor related SRF, increased pigmentation, specific changes in OP and DRPED as well as decreased tumor thickness with an increase in internal reflectivity on ultrasound. Presentation Title: Local tumor control and late complications of fractionated stereotactic radiotherapy in uveal melanoma Authors: Nicole Naus1, Jackelien van Beek1, Caroline van Rij1, Serdar Yavuzyigitoglu1, Dion Paridaens2, Emine Kiliç1 1Erasmus MC , Rotterdam, Netherlands. 2Rotterdam Eye Hospital, Rotterdam, Netherlands Abstract: Purpose: Fractionated stereotactic radiotherapy (fSRT) for uveal melanoma (UM) treats the tumour, while retaining visual function. The aim of our study is to evaluate tumour control, late complications and survival of patients treated with fSRT.Method: We analyzed 184 small to medium-sized uveal melanoma, treated with fSRT from 1999-2014 in Erasmus University Medical Center Rotterdam and Rotterdam Eye Hospital, the Netherlands. We included tumors with at least 4 years of follow up.Results: The mean tumor thickness decreased from 5.9 mm at baseline to 1.9 mm 4 years after fSRT. Tumor progression was observed in 11 of the melanoma patients after a mean of 46 months. Thirty tumors were secondarily enucleated, due to neovascular glaucoma (n=17), tumor progression (n=11) and other reasons (n=2). The most common side effects were radiation retinopathy in 53, vitreous hemorrhage in 38 and neovascular glaucoma in 36 of the patients. The mean disease free survival was 106 months (range 4-218 months) in the metastatic-free group and the mean survival in the group with metastases was 39 months (range 1-165 months) (p<0.001).Conclusion: The local tumor control rate is 94% in uveal melanoma patients treated with fSRT with 15 years of follow up. Date: Saturday 23.3.19 Time: 9:00am 9:45am Session: Free Paper Session: Clinical and Surgical Perspective in Uveal Melanoma Session Chair: Norbert Bornfeld Moderators: Brian Marr and Lauren Dalvin Presentation Title: Vitreo retinal and ocular surgical procedures for the management of intra ocular tumors ocular tumors : A perspective from an ocular oncologist view Authors: Ahmet Sarici Istanbul University-Cerrahpasa, Istanbul, Turkey Abstract: Purpose: To present the ocular and vitreo retinal surgery options intra ocular tumor management Methods: Retrospective chart review of patients between 2010 and 2017. Results: There were 27 of who undergone ocular and vitreo retinal surgeries. 16 patients with intra ocular melanoma undergone pars plana vitrectomy ( 3 had primary tumor endoresection, 4 had endoresection for radiation retinopathy, 4 had pars plana Purpose: To present the ocular and vitreo retinal surgery options intra ocular tumor management Methods: Retrospective chart review of patients between 2010 and 2017. Results: There were 27 of who undergone ocular and vitreo retinal surgeries. 16 patients with intra ocular melanoma undergone pars plana vitrectomy ( 3 had primary tumor endoresection, 4 had endoresection for radiation retinopathy, 4 had pars plana vitrectomy and silicone oil to prevent radiation side effects, 1 had subretinal fluid drainage for exudative retinal detachment, 4 had pars plana vitrectomy and endolaser for management of vitreous hemorrhage ), 3 patients with hemangioblastoma had plana vitrectomy and and endoresection, 5 patients with Coats disease underwent subretinal fluid drainage followed by cryotherapy and endolaser ). Conclusion: Vitreo-retinal surgeries contributes and provides better outcomes in the management of patients. Presentation Title: Features and Incidence of Isolated Choroidal Melanocytosis Authors: Zelia Correa1,2, Cassandra Brooks3, James Augsburger2 1Johns Hopkins Medicine, Wilmer Eye Institute, Baltimore, USA. 2University of Cincinna

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PURPOSE: To test the hypothesis that widely used clinical risk factors for growth of choroidal nevi are associated with malignant transformation. METHODS: Fine needle biopsy for assignment of gene expression profile (class 1 or class 2) was performed in 207 choroidal melanocytic tumors < 3.5 mm in thickness. The class 2 profile was employed as a validated biomarker for malignant transformation. The following data were collected: patient age and sex, tumor diameter and thickness, distance of posterior tumor margin from the optic disc, and the presence or absence of serous retinal detachment, orange lipofuscin pigment, drusen, retinal pigment epithelial fibrosis, retinal pigment epithelial atrophy, visual symptoms, and documented tumor growth. RESULTS: Clinical features associated with the class 2 profile included patient age > 60 years and tumor thickness > 2.25 mm (Fisher exact test, P =.002 for both). Documented growth was not associated with the class 2 profile (P =.5). The odds ratio of a tumor having the class 2 profile was 2.8 (95% confidence interval 1.3-5.9) for patient age > 60 years and 3.5 (95% confidence interval 1.4-8.8) for tumor thickness > 2.25 mm. For patients with both risk factors, the "number needed to treat" to identify 1 patient with a class 2 tumor was 4.3 (P =.0002). No other clinical feature or combination of features was associated with the class 2 profile. CONCLUSIONS: None of the widely used choroidal nevus risk factors for tumor growth, nor documented growth itself, is pathognomonic of malignant transformation as defined by class 2 gene expression profile. Patient age and tumor thickness may be helpful for identifying small choroidal melanocytic tumors that are more likely to have the class 2 profile. Observation for growth prior to treatment continues to be reasonable for most patients with suspicious choroidal nevi. NOTE: Publication of this article is sponsored by the American Ophthalmological Society. (C) 2018 Elsevier Inc. All rights reserved.)

Background: Congenital ocular melanocytosis has been shown to be extremely uncommon in studies of numerous infants and children with retinoblastoma and disorders such as retinopathy of prematurity. Case presentation: A 33-month-old Caucasian boy presented with a solid white predominantly endophytic retinoblastoma filling most of the nasal aspect of the fundus and extensive vitreous seeding. Fundus exam of the contralateral eye showed a broad-based flat melanotic area of the choroid extending from the subfoveal region to the ora serrata temporally. The child was treated by enucleation of the retinoblastoma-containing eye (homozygous non-germline RB1 mutation) and is being monitored annually. The patient has been followed for 4 years. Conclusions: This rare presentation of advanced unilateral retinoblastoma and contralateral isolated choroidal melanocytosis in a young child emphasizes the importance of detailed fundus mapping of the non-affected eye and has potential implications due to the increased incidence of uveal melanoma later in life.

Recognizing that <1% of all uveal melanomas occur in young persons, and that very few clinicians encounter more than a few such cases over an extended career, we felt that a retrospective review of literature and sharing of our clinical experience would be appropriate to remind readers about this age subgroup of patients with posterior uveal melanoma. This interest stems from the increase in reported cases of uveal melanoma in younger individuals and recent advances in the field.

The management of patients with diffuse invasive conjunctival melanoma focuses on local tumor control and screening for metastasis. Despite the lack of consensus on the benefit of sentinel lymph node biopsy for these neoplasms, the information obtained by histopathology is useful for tumor staging and treatment planning. Due to the lack of evidence of survival improvement, orbital exenteration is being performed with diminishing frequency. We describe a patient with diffuse invasive conjunctival melanoma and lymph node involvement treated by tumor debulking, brachytherapy (custom unshielded radioactive device), and adjuvant ipilimumab who has had a favorable outcome without emergence of local tumor relapse or distant metastasis during 16 months of follow up.

PURPOSETo evaluate the incidence, potential correlation with transcleral fine needle aspiration biopsy, and treatment of scleral necrosis in patients with posterior uveal melanomas treated by 125I plaque radiotherapy and assessed by transcleral fine needle aspiration biopsy.METHODSWe per-formed a retrospective review of posterior uveal melanoma treated by 125I plaque radiotherapy at a single academic institution between July 2006 and July 2013. Consecutive patients diagnosed with a posterior uveal melanoma during the study period that had an anterior margin at or anterior to the equator who were evaluated by transcleral fine needle aspiration biopsy prior to 125I plaque radiotherapy were included. The main outcome measure was development of scleral necrosis, and the secondary outcome was treatment of this complication. Statistical analysis included computation of conventional descriptive statistics, cross-tabulation and chi-square tests of potential factors related to the development of scleral necrosis, and summarizing of treatment approaches and results. The incidence of treatment of scleral necrosis was calculated using the Kaplan-Meier method.RESULTSDuring the 7-year study period, 87 posterior uveal melanomas were evaluated by transcleral fine needle aspiration biopsy and treated by 125I plaque radiotherapy. The median largest basal diameter of the tumor was 13.3 mm, and the median thickness was 6.8 mm. Eight patients (9.2%) developed scleral necrosis during follow-up. Thicker tumors (> 6.5 mm) were more likely to develop scleral necrosis (n=7) than thinner tumors (p=0.05). The median interval between 125I plaque radiotherapy and detection of scleral necrosis was 19.1 months. The overall cumulative probability of scleral necrosis was 6.2% at 6 months and 14.3% at 24 months, subsequently remaining stable. For thicker tumors, the probability of scleral necrosis was 23.5% at 45.4 months. Five patients were treated by scleral patch graft (62.5%) and three by observation (37.5%). One patient underwent enucleation after two failed scleral patch attempts and recurrent scleral necrosis. The mean follow-up period for patients with scleral necrosis was 34.5 months.CONCLUSIONSThicker posterior uveal melanomas are more likely to develop scleral necrosis after 125I plaque radiotherapy and transcleral fine needle aspiration biopsy. While observation is sufficient for managing limited scleral necrosis, scleral patch graft is a viable alternative for eye preservation in extensive scleral necrosis.

Repetitive intravitreal injections of Methotrexate (MTX), a hydrophilic chemotherapeutic drug, are currently used to treat selected vitreoretinal (VR) diseases, such as intraocular lymphoma. To avoid complications associated with the rapid release of MTX from the injections, a Polylactic acid (PLA) and Chitosan (CS)-based MTX micro-implant prototype was fabricated in an earlier study, which showed a sustained therapeutic release rate of 0.2-2.0 mu g/day of MTX for a period similar to 1 month in vitro and in vivo. In the current study, different combinations of Poly(lactic-co-glycolic) acid (PLGA)/PLA coatings were used for lipophilic surface modification of the CS-MTX micro-implant, such as PLGA 5050, PLGA 6535 and PLGA 7525 (PLA: PGA - 50:50, 65:35, 75:25, respectively; M.W: 54,400 - 103,000) and different PLA, such as PLA 100 and PLA 250 (MW: 102,000 and 257,000, respectively). This improved the duration of total MTX release from the coated CS-MTX micro-implants to similar to 3-5 months. With an increase in PLA content in PLGA and molecular weight of PLA, a) the initial burst of MTX and the mean release rate of MTX can be reduced; and b) the swelling and biodegradation of the micro-implants can be delayed. The controlled drug release mechanism is caused by a combination of diffusion process and hydrolysis of the polymer coating, which can be modulated by a) PLA content in PLGA and b) molecular weight of PLA, as inferred from Korsmeyer Peppas model, Zero order, First order and Higuchi model fits. This improved micro-implant formulation has the potential to serve as a platform for controlled release of hydrophilic drugs to treat selected VR diseases.

Importance:Given the rarity of posterior uveal melanoma in patients younger than 21 years, reporting clinical experience in this area has relevance.Objective:To describe the baseline clinical features, treatment, and clinical course of a group of patients younger than 21 years who have primary posterior uveal melanoma.Design, Setting, and Participants:This retrospective descriptive case series of patients younger than 21 years who have a primary choroidal or ciliochoroidal melanoma was conducted at a single-center subspecialty referral practice. Patients in the relevant age group who were treated in a single practice between July 1980 and December 2013 were included; clinical data collected through December 2017 were captured to permit adequate follow-up time in all cases.Main Outcomes and Measures:Conventional descriptive statistics of relevant clinical variables (eg, demographic, tumor, treatment, and outcome variables) of each patient were recorded. Actuarial metastasis-free and overall survival curves were computed and plotted, as was a postdetection survival curve of patients who developed metastasis during available follow-up.Results:Of 2265 patients with posterior uveal melanoma encountered by the authors during the study interval, 18 (0.8%) were younger than 21 years when diagnosed and treated. Ten were female and 8 male, and the mean (SD) age was 16.6 (4.2) years. Through available follow-up, 8 of these patients had developed metastatic uveal melanoma (44%). All 8 died of metastasis. Actuarial survival analysis showed that the cumulative probability of metastatic death in this group exceeded 50%. The median overall survival time after treatment of the primary intraocular tumor was 11.9 (95% CI, 7.3-16.5) years. The median survival time after detection of metastasis in the 8 patients who developed metastasis was 2.3 months (95% CI, 0.0-5.2) months.Conclusions and Relevance:Posterior uveal melanoma in patients younger than 21 years appears to have a similar if not worse prognosis than patients with PUM in the population overall. Owing to the later onset of metastasis observed, patients younger than 21 years should continue to have surveillance tests for more than 10 years after treatment.