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      Retinal Pigment Epithelium Adenoma and Adenocarcinoma: A Review


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          Adenoma and adenocarcinoma of the retinal pigment epithelium (RPE) are rare intraocular tumours that are often misdiagnosed as posterior uveal melanoma or other simulating lesions. It is important to be able to differentiate these tumours from melanoma for 3 reasons. First, an inability to rule out melanoma often tilts the management towards enucleation. Second, management options like radiotherapy and local resection which work well for melanoma may not be easily applied to these tumours. Third, and most importantly, patients with melanoma need a lifetime follow-up to rule out metastases (metastatic dormancy) whereas RPE tumours hardly metastasize. An abruptly elevated, often deeply pigmented tumour, with a prominent retinal feeding artery and a draining vein causing exudation, should raise a suspicion of RPE tumours. RPE tumours have a remarkable local invasive potential but a low metastatic potential. Most RPE tumours require treatment due to local complications. Small, asymptomatic tumours can be generally observed. Enucleation is still the gold standard of treatment, although local resection has been reported in selected cases with good results. Here, we provide a comprehensive review of the demographic, clinical, and imaging features of true acquired neoplasms of the RPE, namely adenoma and adenocarcinoma, the ways to differentiate them from melanoma, their clinical course and prognosis, and Options for their management.

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          Most cited references49

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          Peripheral exudative hemorrhagic chorioretinopathy simulating choroidal melanoma in 173 eyes.

          To evaluate the clinical features and outcomes of eyes with peripheral exudative hemorrhagic chorioretinopathy (PEHCR) simulating choroidal melanoma.
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            Micrometastatic Dormancy in Uveal Melanoma: A Comprehensive Review of the Evidence, Mechanisms, and Implications for Future Adjuvant Therapies

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              Solitary congenital hypertrophy of the retinal pigment epithelium: clinical features and frequency of enlargement in 330 patients.

              To describe the clinical features of solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE) and to determine the frequency of enlargement of this lesion Retrospective, observational, noncomparative case series. Three hundred thirty consecutive patients with solitary CHRPE. The 3 main outcome measures included flat lesion enlargement, intralesional lacunae enlargement, and development of an elevated nodule within the lesion. The clinical features at the time of presentation were analyzed for their impact on the main outcomes using a series of Cox proportional hazards regressions. The most common referring diagnosis included choroidal nevus (26%), choroidal melanoma (15%), CHRPE (9%), and unspecified lesion (48%). The median age at diagnosis was 45 years (range, 1-80 years), and there were no patients with familial adenomatous polyposis or related colon cancer, although a history of cancer was noted in 8% of patients, most commonly breast cancer (3%). The lesion most frequently was located inferotemporally (31%) and at the equatorial region (45%). Rarely, it was located in the macula (1%) or peripapillary region (1%). The median largest basal diameter was 4.5 mm, and the lesion was flat in all cases except in 5 (1.5%), in which there was an intralesional lesion nodule. The lesion was pigmented in 88% of cases and nonpigmented in 12%. Lacunae were noted in 43% of the pigmented CHRPE, and the lacunae showed gradual enlargement in 32%. Factors related to lacunae enlargement included number and relative size of lacunae. Flat enlargement of the lesion was documented in 46% of patients with comparative photographic follow-up and in 83% of those followed up for more than 3 years. The median rate of enlargement was 10 micro m per month. The most important factor associated with flat lesion enlargement was relative size of the lacunae within CHRPE. There were no cases of CHRPE in which a nodule developed while the patient was being followed up. Of the 5 lesions that had a nodule, progressive enlargement of the nodule was found in 3. Congenital hypertrophy of the retinal pigment epithelium generally has been regarded as a benign, stable lesion, but subtle, flat enlargement was noted in most patients (83%) followed up for 3 or more years using meticulous photographic comparison. Flat enlargement of the lesion appeared to be related to percentage of the mass occupied by lacunae.

                Author and article information

                Ocular Oncology and Pathology
                S. Karger AG
                March 2021
                22 December 2020
                : 7
                : 2
                : 121-132
                [_a] aShri Bhagwan Mahavir Vitreoretinal Services, Chennai, India
                [_b] bL&T Ocular Pathology Department, Vision Research Foundation, Chennai, India
                Author notes
                *Pukhraj Rishi, Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18 College Road, Chennai 600006 (India), docrishi@yahoo.co.in
                509484 Ocul Oncol Pathol 2021;7:121–132
                © 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.

                : 23 May 2020
                : 16 June 2020
                Page count
                Figures: 3, Tables: 2, Pages: 12
                Clinical Research

                Vision sciences,Ophthalmology & Optometry,Pathology
                Malignancy,Eye,Retina,Retinal pigment epithelium,Uveal melanoma,Biopsy,Tumour,Adenoma,Pathology,Enucleation,Adenocarcinoma


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