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      High-Risk Histopathologic Features of Retinoblastoma Treated at a Tertiary Hospital in West Java, Indonesia

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          Abstract

          Purpose: To evaluate the histopathological characteristics of clinically advanced retinoblastoma (RB) and its relationship with tumor differentiation. Methods: This was a cross-sectional study of primary enucleated group D/E intraocular RB using medical records from 2017 to 2020 in a tertiary referral hospital. Cases with incomplete histopathological results were excluded. Tumors were classified into well, moderately, and poorly differentiated and undifferentiated. High-risk histopathological features were classified as per Thaung and Karaa [ Community Eye Health. 2018;31(101):17–3]. Results: This study included 121 patients (129 eyes), of which 32.2% were diagnosed at 25–36 months. High-risk features (HRFs) were found in 100/129 eyes, and of 73 complete histopathological results, the 2 most common HRFs were postlaminar optic nerve invasion and massive choroidal invasion. RB was poorly differentiated in 69.9% and well differentiated in 12.3% of eyes. There was no statistically significant association between any HRFs and tumor differentiation, with age >2 years associated with tumor differentiation ( p < 0.05). Conclusion: The frequency of HRFs is 77.5% of primary enucleated eyes, mainly poorly and undifferentiated cells, particularly in children aged >2 years old.

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          Most cited references40

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          Retinoblastoma in Asia

          Asia-Pacific region bears a significant global burden of retinoblastoma (RB), therefore understanding RB in Asia-Pacific region is important. Based on the year 2013 population estimates, 43% (3452 of 8099 children) of the global burden of RB lives in 6 countries of Asia-Pacific region: 1486 children in India, 1103 children in China, 277 children in Indonesia, 260 children in Pakistan, 184 children in Bangladesh, 142 children in Philippines. There exists a wide disparity, technological and socio-economical, within countries in this region resulting in a varied pattern of clinical presentation and survival varies. Challenges in developing nations are not just technological, but also social. Opportunities emerge for research to study and understand the socio-economical aspects of the disease to develop interventions that are relevant culturally and feasible economically. Possible steps include disease education and counselling, universal screening, highly subsidized/free of cost treatment for low socioeconomic strata, raising funds through the government and non-governmental organizations, sensitization and training of man-power in screening, diagnosis and treatment, and developing new specialized centers with tele-ophthalmology services.
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            Is Open Access

            Retinoblastoma: Etiology, Modeling, and Treatment

            Retinoblastoma is a retinal cancer that is initiated in response to biallelic loss of RB1 in almost all cases, together with other genetic/epigenetic changes culminating in the development of cancer. RB1 deficiency makes the retinoblastoma cell-of-origin extremely susceptible to cancerous transformation, and the tumor cell-of-origin appears to depend on the developmental stage and species. These are important to establish reliable preclinical models to study the disease and develop therapies. Although retinoblastoma is the most curable pediatric cancer with a high survival rate, advanced tumors limit globe salvage and are often associated with high-risk histopathological features predictive of dissemination. The advent of chemotherapy has improved treatment outcomes, which is effective for globe preservation with new routes of targeted drug delivery. However, molecularly targeted therapeutics with more effectiveness and less toxicity are needed. Here, we review the current knowledge concerning retinoblastoma genesis with particular attention to the genomic and transcriptomic landscapes with correlations to clinicopathological characteristics, as well as the retinoblastoma cell-of-origin and current disease models. We further discuss current treatments, clinicopathological correlations, which assist in guiding treatment and may facilitate globe preservation, and finally we discuss targeted therapeutics for future treatments.
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              High-risk retinoblastoma based on international classification of retinoblastoma: analysis of 519 enucleated eyes.

              To determine the correlation between the International Classification of Retinoblastoma (ICRB) and histopathologic high-risk retinoblastoma.
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                Author and article information

                Journal
                OOP
                OOP
                10.1159/issn.2296-4657
                Ocular Oncology and Pathology
                S. Karger AG
                2296-4681
                2296-4657
                2021
                October 2021
                20 July 2021
                : 7
                : 5
                : 353-360
                Affiliations
                [_a] aDepartment of Child Health, Dr. Hasan Sadikin Hospital/Universitas Padjadjaran, Bandung, Indonesia
                [_b] bFaculty of Medicine, Universitas Padjadjaran, Bandung, Indonesia
                [_c] cDepartment of Opthalmology, Cicendo National Eye Hospital, Universitas Padjadjaran, Bandung, Indonesia
                [_d] dDepartment of Anatomic Pathology, Dr. Hasan Sadikin Hospital, Universitas Padjadjaran, Bandung, Indonesia
                [_e] eAnatomic Pathology Laboratory, Cicendo National Eye Hospital, Universitas Padjadjaran, Bandung, Indonesia
                Article
                517100 Ocul Oncol Pathol 2021;7:353–360
                10.1159/000517100
                c263644f-6e6b-4ba0-ba8f-088c70247a1e
                © 2021 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
                : 26 January 2021
                : 07 May 2021
                Page count
                Figures: 1, Tables: 4, Pages: 8
                Categories
                Research Article

                Vision sciences,Ophthalmology & Optometry,Pathology
                Retinoblastoma,Age,Developing countries,High-risk pathology features,Tumor differentiation

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