12
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      Are you tired of sifting through news that doesn't interest you?
      Personalize your Karger newsletter today and get only the news that matters to you!

      Sign up

      • Record: found
      • Abstract: found
      • Article: found

      Conjunctival Melanoma in Childhood and Adolescence: A Systematic Review

      review-article

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Background: Conjunctival melanoma is rare in adults and rarer in children. We systematically reviewed the presentation, diagnostic and management strategies as well as outcomes for conjunctival melanoma in children and adolescents. Methods: The following databases were searched: Medline, Embase, Web of Science and Scopus for cases of conjunctival melanoma occurring in children and adolescents < 18 years of age. Results: Seventeen studies with 32 patients (18 males) were identified. The median age at presentation was 11 years (range 4–18 years). Most patients were white. Most patients presented with a conjunctival mass or naevus with a recent history of growth or change. Excision biopsy provided diagnosis and management for all cases. Adjuvant chemotherapy and radiotherapy were also used. One patient had metastatic disease at diagnosis and 3 developed metastatic disease (range 1–10 months). Two patients died from disease and one was alive with metastatic disease. Two patients had disease recurrence. Outcomes were observed to be better where diagnosis was made earlier and “no-touch” excision biopsy was performed in an appropriate specialist setting. Conclusions: Conjunctival melanoma occurs rarely in children and adolescents. Surgery is the mainstay of management. The prognosis is guarded in metastatic disease due to the small sample size and limited follow-up.

          Related collections

          Most cited references34

          • Record: found
          • Abstract: found
          • Article: not found

          Binimetinib versus dacarbazine in patients with advanced NRAS-mutant melanoma (NEMO): a multicentre, open-label, randomised, phase 3 trial.

          There are no established therapies specific for NRAS-mutant melanoma despite the emergence of immunotherapy. We aimed to assess the efficacy and safety of the MEK inhibitor binimetinib versus that of dacarbazine in patients with advanced NRAS-mutant melanoma.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Conjunctival melanomas harbor BRAF and NRAS mutations and copy number changes similar to cutaneous and mucosal melanomas.

            Conjunctival melanoma is a rare but potentially deadly tumor of the eye. Despite effective local therapies, recurrence and metastasis remain frequent. Once the tumor has metastasized, treatment options are limited and the prognosis is poor. To date, little is known of the genetic alterations in conjunctival melanomas. We conducted genetic analysis of 78 conjunctival melanomas, to our knowledge the largest cohort reported to date. An oncogene hotspot array was run on 38 samples, screening for a panel of known cancer-relevant mutations. Thirty tumors were analyzed for genome-wide copy number alterations (CNA) using array-based comparative genomic hybridization. Sanger sequencing of selected target mutations was conducted in all samples. BRAF mutations were identified in 23 of 78 (29%) tumors. NRAS mutations, previously not recognized as relevant in conjunctival melanoma, were detected in 14 of 78 (18%) tumors. We found CNAs affecting various chromosomes distributed across the genome in a pattern reminiscent of cutaneous and mucosal melanoma but differing markedly from uveal melanoma. The presence of NRAS or BRAF mutations in a mutually exclusive pattern in roughly half (47%) of conjunctival melanomas and the pattern of CNAs argue for conjunctival melanoma being closely related to cutaneous and mucosal melanoma but entirely distinct from uveal melanoma. Patients with metastatic conjunctival melanoma should be considered for therapeutic modalities available for metastatic cutaneous and mucosal melanoma including clinical trials of novel agents.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Conjunctival Tumors: Review of Clinical Features, Risks, Biomarkers, and Outcomes--The 2017 J. Donald M. Gass Lecture.

              Conjunctival tumors encompass a broad range of diagnoses. The 3 most important malignant tumors include ocular surface squamous neoplasia (OSSN) (14%), melanoma (12%), and lymphoma (7%). Conjunctival malignancies are rarely found in children. Regarding OSSN, pre-disposing conditions include chronic solar radiation, immune deficiency (HIV), organ transplant, autoimmune conditions, xeroderma pigmentosum, and chronic exposure to cigarette smoke. OSSN is managed surgically or with topical/injection immunotherapy or chemotherapy. Metastasis occurs in 2 mm thickness or those located in fornix, caruncle, or orbit are at highest risk for metastasis. Regarding lymphoma, predisposing conditions include benign reactive lymphoid hyperplasia, immune deficiency (HIV), immune dysfunction, and chronic inflammation/infection (Helicobacter pylori, Chlamydia psittaci). The 4 most important subtypes include extranodal marginal zone lymphoma (ENMZL), follicular lymphoma, mantle cell lymphoma (MCL), and diffuse large B-cell lymphoma. Treatment includes surgical resection, cryotherapy, radiotherapy, systemic chemotherapy, or targeted anti-B-cell therapy (rituximab). Lymphoma-related survival (5-year) depends on subtype and ranges from 97% (ENMZL) to 9% (MCL). Recognizing conjunctival tumors and understanding predisposing factors, biomarkers, and treatment strategies are vital to patient outcomes.
                Bookmark

                Author and article information

                Journal
                OOP
                OOP
                10.1159/issn.2296-4657
                Ocular Oncology and Pathology
                S. Karger AG
                2296-4681
                2296-4657
                2019
                October 2019
                10 April 2019
                : 5
                : 6
                : 387-395
                Affiliations
                [_a] aSydney Children’s Hospital, Randwick, New South Wales, Australia
                [_b] bSchool of Women’s and Children’s Health, University of New South Wales, Randwick, New South Wales, Australia
                [_c] cSt. Vincent’s Hospital, Darlinghurst, New South Wales, Australia
                [_d] dSave Sight Institute, University of Sydney, Sydney, New South Wales, Australia
                [_e] eSydney Eye Hospital, Sydney, New South Wales, Australia
                [_f] fKid’s Cancer Centre, Sydney Children’s Hospital, Randwick, New South Wales, Australia
                [_g] gNelune Comprehensive Cancer Centre, Prince of Wales Hospital, Randwick, New South Wales, Australia
                Author notes
                *Antoinette C. Anazodo, Kid’s Cancer Centre, Sydney Children’s Hospital, High Street, Randwick, NSW 2031 (Australia), E-Mail antoinette.anazodo@health.nsw.gov.au
                Author information
                https://orcid.org/0000-0003-4697-3820
                Article
                497813 PMC6873081 Ocul Oncol Pathol 2019;5:387–395
                10.1159/000497813
                PMC6873081
                31768361
                a03bc2b4-3488-4d85-aa9a-25ca6626517e
                © 2019 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.

                History
                : 16 November 2018
                : 08 February 2019
                Page count
                Figures: 1, Tables: 1, Pages: 9
                Categories
                Review Article

                Vision sciences,Ophthalmology & Optometry,Pathology
                Conjunctival melanoma,Paediatric ophthalmology,Paediatric oncology

                Comments

                Comment on this article