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      Incidental Diagnosis of Four Lid Orbital Lymphoma during a Blepharoplasty


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          Chronic lymphocytic leukemia is characterized by the accumulation of mature CD5-positive B-cells in the lymphoid organs. 1 Extranodal involvement occurs in up to 10% of cases and can arise in various tissues, including the orbit. Less than 400 cases of orbital lymphoma are diagnosed per year in the United States, typically manifesting as a form of B-cell non-Hodgkin lymphoma, with extranodal marginal zone B-cell lymphoma being the most common subtype. Orbital lymphoma typically presents with proptosis and a palpable mass; however, patients may also have a relatively benign examination. Here, we present a 76-year-old man with symmetric dermatochalasis and marked fat prolapse of all four lids, who was incidentally diagnosed with secondary orbital lymphoma in all four eyelids during a cosmetic four lid blepharoplasty. His history was significant for RAI Stage 0 chronic lymphocytic leukemia diagnosed 15 years before consultation. Orbital lymphoma presenting as orbital fat prolapse has only been reported a few times in the literature. To our knowledge, this is the first case of secondary orbital lymphoma in all four eyelids found incidentally during an aesthetic four lid blepharoplasty.

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          Incidence of orbital, conjunctival and lacrimal gland malignant tumors in USA from Surveillance, Epidemiology and End Results, 1973-2009.

          To determine the types and incidence of tumors affecting the orbit, conjunctiva and lacrimal glands and to study the trend line of these tumors in the United States from 1973 to 2009.
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            Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma.

            Lymphomas of the orbit and eye are rare conditions that should be treated as separate entities due to the differences in presumed aetiology, investigations, management and outcomes. Orbital lymphoma is most often of low-grade histology; thyroid eye disease may predispose and chlamydial infection has been suggested as a trigger. Commonly, stage IE, in most cases, can be managed with radiotherapy alone using either a kilovoltage portal for conjunctival disease or a wedged pair of megavoltage beams for more infiltrative disease to a dose of 30 Gy in 15 fractions over 3 weeks. However, medical therapy is being investigated, including a rituximab-only approach for conjunctival-only presentations. The cure rate for stage IE disease is very high. In contrast, primary ocular lymphoma is often of high-grade histology, in particular diffuse large B-cell lymphoma, and can be regarded as one end of primary central nervous system lymphoma - both eyes and brain being at risk. Immunosuppression predisposes to the disease. Management consists of an initial high-dose chemotherapy regimen with methotrexate. In most cases, this should be followed by radiotherapy to the whole brain and globes to a dose of 30-36 Gy with a boost to bulk/presenting disease. Cure rates are rarely above 50%.
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              Chronic lymphocytic leukemia of the orbit.


                Author and article information

                Plast Reconstr Surg Glob Open
                Plast Reconstr Surg Glob Open
                Plastic and Reconstructive Surgery Global Open
                Lippincott Williams & Wilkins (Hagerstown, MD )
                June 2024
                06 June 2024
                : 12
                : 6
                : e5870
                From the [* ]Division of Plastic and Reconstructive Surgery, Department of Surgery, Corewell Health Michigan State University Plastic Surgery Residency, Grand Rapids, Mich.
                []Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Colorado, Anschutz Medical Campus, Aurora, Colo.
                []Physician Assistant Studies Program, Trine University, Fort Wayne, Ind.
                [§ ]Eye Plastic and Facial Cosmetic Surgery, Grand Rapids, Mich.
                Author notes
                Brandt A. Gruizinga, MD, 80 Clear Creek Lane, #11, Golden, CO 80403, E-mail: brandt.gruizinga@ 123456cuanschutz.edu
                GOX-D-24-00018 00017
                Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                : 5 January 2024
                : 28 March 2024
                Funded by: Not Applicable
                Award ID: Not Applicable
                Award Recipient : Not Applicable
                Case Report
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                UNITED STATES


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