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      Multiple Uveal Melanoma


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          Introduction: Multiple uveal melanoma is a rare occurrence and includes bilateral melanoma, unilateral multiple/multifocal melanoma, or melanoma with metastasis to the ipsilateral or contralateral eye. Methods: A chart review of patients diagnosed with uveal melanoma between January 2013 and January 2019 at the Duke University Eye Center Ophthalmic Oncology Service was performed. Results: Three patients with multiple, sequential melanoma were identified; patient 1 had bilateral choroidal melanoma and patients 2 and 3 had 2 choroidal melanomas occurring in the same eye. In all 3 patients, both the first and sequential choroidal melanomas were treated with I-125 radioactive plaque brachytherapy (PBT). Two patients were found to have developed secondary metastatic uveal melanoma as a presenting sign of systemic metastases. Patient 4, initially treated with PBT, was diagnosed with ipsilateral metastatic choroidal melanoma, also treated with PBT. Patient 5 had right eye enucleation for choroidal melanoma and developed vision-threatening metastasis in the left eye, which was treated with PBRT. None of the patients had history of cancer prior to their first diagnosis. Patients 1 and 5 were tested with a systemic melanoma panel; both were negative for BAP1, but patient 1 had a variant of unknown significance in BRCA2. Patient 3 had oculodermal melanocytosis, an established risk factor of uveal melanoma. Conclusion: Although rare, the possibility of multiple uveal melanoma does exist. Examination of the treated and contralateral eye on a regular basis is crucial, not only to identify local failure but also new metastases from the primary tumor and additional primary tumors.

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          Uveal Melanoma: 5-Year Update on Incidence, Treatment, and Survival (SEER 1973-2013)

          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.
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            Is Open Access

            Trends in incidence, survival, and management of uveal melanoma: a population-based study of 7,516 patients from the Surveillance, Epidemiology, and End Results database (1973–2012)

            Introduction Uveal melanoma (UM) is the most common primary intraocular malignancy, despite comprising 50 years (OR 4.0, CI =3.4–4.6), distant metastases (OR 8.6, CI =4.7–15), and primary surgical treatment (OR 2.6, CI =2.0–3.3) as independently associated with increased mortality, P<0.005. Conversely, patients identified as Hispanic (OR 0.6, CI =0.5–0.8) and patients receiving radiation treatment (OR 0.5, CI =0.4–0.7) were independently associated with reduced mortality, P<0.005. Conclusion UM remains a rare form of melanoma that occurs primarily in Caucasian patients older than 50 years. More than two-thirds of UM patients are curatively treated with primary radiotherapy as opposed to surgery, which has resulted in a significant improvement in both overall survival and cancer-specific survival. Despite this shift in management strategy, the mean 5-year cancer-specific survival rate remained relatively unchanged during the study period. Male sex, older age, distant disease, and primary surgical therapy rather than radiotherapy are associated with an increased risk of mortality.
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              Clinical Characteristics of Uveal Melanoma in Patients With Germline BAP1 Mutations.

              Somatic mutations in BAP1 (BRCA1-associated protein 1 gene) are frequently identified in uveal melanoma. To date, the role of germline BAP1 mutations in uveal melanoma has not been characterized.

                Author and article information

                Ocular Oncology and Pathology
                S. Karger AG
                October 2020
                15 July 2020
                : 6
                : 5
                : 368-375
                Duke Eye Center, Duke University School of Medicine, Durham, North Carolina, USA
                Author notes
                *Miguel Angel Materin, Duke Eye Center, Duke University School of Medicine, 2351 Erwin Road, Durham, NC 27705 (USA), miguel.materin@duke.edu
                Author information
                508393 Ocul Oncol Pathol 2020;6:368–375
                © 2020 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                : 18 February 2020
                : 30 April 2020
                Page count
                Figures: 4, Tables: 1, Pages: 8
                Research Article

                Vision sciences,Ophthalmology & Optometry,Pathology
                Unilateral multiple choroidal/uveal melanoma,Oculodermal melanocytosis, BRCA2 ,Bilateral choroidal/uveal melanoma, BAP1 ,Contralateral metastasis,Unilateral multifocal choroidal/uveal melanoma,Interocular metastasis,Intraocular metastasis


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