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      Occult anterior uveal melanomas presenting as extrascleral extension

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          Abstract

          Objective

          To describe the management of patients with occult anterior uveal melanomas presenting with extrascleral extension.

          Methods and analysis

          Retrospective case series including five patients with small pigmented nodular mass on the episclera. Each lesion was documented by slit-lamp photography and measured with high-frequency ultrasound imaging (ultrasound biomicroscopy). Diagnosis of uveal melanoma was confirmed by biopsy with lamellar sclerectomy. Immediate scleral patch graft repair was performed. Later, each tumour was treated with palladium-103 ophthalmic plaque brachytherapy. The mean plaque diameter was 12 mm (median, 12; range, 10–14). A mean apex prescription dose of 87 Gy (median, 84.5; range, 82.3–99.2) to a tumour depth of 2 mm from the inner sclera delivered over 7 continuous days. The main outcome measures were best-corrected visual acuity, changes in tumour and scleral characteristics and complications.

          Results

          During each surgery, residual tumour was visualised within an emissary passageway at the deep plane of scleral resection. At a mean of 80 months (median, 57; range, 24–159) follow-up, no patients experienced graft infection, scleromalacia or rejection. Biopsy was required to establish the diagnosis, transillumination failed, and therefore ultrasound measurements were used to determine the plaque size required to treat the relatively occult intraocular component. Despite these challenges, there were no cases of local tumour recurrence, secondary enucleation or metastatic disease. Attributed to cataract surgery, visual acuities improved in three patients and two were stable.

          Conclusion

          Extrascleral uveal melanoma extension can occur with undetectable, occult intraocular tumours. In these cases, plaque radiation effectively induced local tumour control, preserved vision and prevented metastasis.

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          Most cited references34

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          Uveal melanoma: From diagnosis to treatment and the science in between.

          Melanomas of the choroid, ciliary body, and iris of the eye are collectively known as uveal melanomas. These cancers represent 5% of all melanoma diagnoses in the United States, and their age-adjusted risk is 5 per 1 million population. These less frequent melanomas are dissimilar to their more common cutaneous melanoma relative, with differing risk factors, primary treatment, anatomic spread, molecular changes, and responses to systemic therapy. Once uveal melanoma becomes metastatic, therapy options are limited and are often extrapolated from cutaneous melanoma therapies despite the routine exclusion of patients with uveal melanoma from clinical trials. Clinical trials directed at uveal melanoma have been completed or are in progress, and data from these well designed investigations will help guide future directions in this orphan disease. Cancer 2016;122:2299-2312. © 2016 American Cancer Society.
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            The American Brachytherapy Society consensus guidelines for plaque brachytherapy of uveal melanoma and retinoblastoma.

            (2014)
            To present the American Brachytherapy Society (ABS) guidelines for plaque brachytherapy of choroidal melanoma and retinoblastoma.
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              The biology of uveal melanoma

              Uveal melanoma (UM), a rare cancer of the eye, is distinct from cutaneous melanoma by its etiology, the mutation frequency and profile, and its clinical behavior including resistance to targeted therapy and immune checkpoint blockers. Primary disease is efficiently controlled by surgery or radiation therapy, but about half of UMs develop distant metastasis mostly to the liver. Survival of patients with metastasis is below 1 year and has not improved in decades. Recent years have brought a deep understanding of UM biology characterized by initiating mutations in the G proteins GNAQ and GNA11. Cytogenetic alterations, in particular monosomy of chromosome 3 and amplification of the long arm of chromosome 8, and mutation of the BRCA1-associated protein 1, BAP1, a tumor suppressor gene, or the splicing factor SF3B1 determine UM metastasis. Cytogenetic and molecular profiling allow for a very precise prognostication that is still not matched by efficacious adjuvant therapies. G protein signaling has been shown to activate the YAP/TAZ pathway independent of HIPPO, and conventional signaling via the mitogen-activated kinase pathway probably also contributes to UM development and progression. Several lines of evidence indicate that inflammation and macrophages play a pro-tumor role in UM and in its hepatic metastases. UM cells benefit from the immune privilege in the eye and may adopt several mechanisms involved in this privilege for tumor escape that act even after leaving the niche. Here, we review the current knowledge of the biology of UM and discuss recent approaches to UM treatment.
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                Author and article information

                Journal
                Br J Ophthalmol
                Br J Ophthalmol
                bjophthalmol
                bjo
                The British Journal of Ophthalmology
                BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                0007-1161
                1468-2079
                November 2023
                1 September 2022
                : 107
                : 11
                : 1698-1703
                Affiliations
                [1 ] departmentOcular Tumor, Orbital Disease, and Ophthalmic Radiation Therapy , The New York Eye Cancer Center , New York, NY, USA
                [2 ] New York Eye Cancer Center , New York, New York, USA
                [3 ] departmentPathology and Laboratory Science , New York Eye and Ear Infirmary of Mount Sinai , New York, New York, USA
                Author notes
                [Correspondence to ] Dr Paul T Finger, Ocular Tumor, Orbital Disease, and Ophthalmic Radiation Therapy, The New York Eye Cancer Center, New York, New York 10065, USA; pfinger@ 123456eyecancer.com
                Author information
                http://orcid.org/0000-0002-8111-3896
                Article
                bjophthalmol-2022-321837
                10.1136/bjo-2022-321837
                10646849
                36126107
                8687f441-601e-42e4-bd68-0f5a340d9846
                © Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY. Published by BMJ.

                This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See: https://creativecommons.org/licenses/by/4.0/.

                History
                : 20 May 2022
                : 17 August 2022
                Funding
                Funded by: The Eye Cancer Foundation;
                Award ID: N/A
                Categories
                Clinical Science
                1506
                Custom metadata
                unlocked

                Ophthalmology & Optometry
                choroid,diagnostic tests/investigation,neoplasia,pathology
                Ophthalmology & Optometry
                choroid, diagnostic tests/investigation, neoplasia, pathology

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