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Observations: A 42-year-old woman developed rapid central functional decline to light perception with optic disc swelling and progressive exudative retinal detachment. Diagnostic vitrectomy showed a monoclonal B-cell population with elevated interleukin-10/-6 and immunoglobulin heavy-chain clonality. Intravitreal methotrexate was started the next day, leading to marked visual restoration to 20/20 and anatomical resolution. Conclusions and importance: Fulminant optic disc involvement with extensive serous retinal detachment can still recover with timely diagnosis and treatment.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/rapidly-progressive-primary-vitreoretinal-lymphoma-with-optic-disc-involvement-and-retinal-detachment/432198/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/rapidly-progressive-primary-vitreoretinal-lymphoma-with-optic-disc-involvement-and-retinal-detachment/432198.png","ImageObject",300,407,{"name":92,"@type":93},"Mabel","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-29",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",13,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What condition was ultimately diagnosed in the report?","Question",{"text":113,"@type":114},"The final diagnosis was primary vitreoretinal lymphoma (PVRL), confirmed by diagnostic vitrectomy with cytopathology, flow cytometry, cytokine analysis, and molecular studies showing clonality.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How did the patient’s vision change after treatment?",{"text":118,"@type":114},"After prompt intravitreal methotrexate, vision improved dramatically from light perception to 20/20, along with resolution of the central scotoma, vitreous opacity, and optic disc swelling.",{"name":120,"@type":111,"acceptedAnswer":121},"Why was diagnosis difficult in this case?",{"text":122,"@type":114},"PVRL presented as a diagnostic mimic resembling infectious retinitis or posterior uveitis, worsening rapidly despite empiric antimicrobials, which delayed definitive identification until invasive diagnostic vitrectomy.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},432198,1791164591,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":29,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},7971474920318,"https://ap-avatar.wpscdn.com/avatar/74000ee537e6b0ca360?x-image-process=image/resize,m_fixed,w_180,h_180&k=1788430171632181674","American Journal of Ophthalmology Case Reports 41 (2026) 102496  \nContents lists available at ScienceDirect  \nAmerican Journal of Ophthalmology Case Reports  \njournal [homepage:](homepage: www.ajocasereports.com/)[ www.ajocasereports.com/](homepage: www.ajocasereports.com/)  \n| Rapidly progressive primary vitreoretinal lymphoma with optic disc involvement and retinal detachment\u003Cbr>*\u003Cbr>Masahiro Akada , Yuki Muraoka , Kenji Ishihara , Shin Kadomoto , Masayuki Hata , Akitaka Tsujikawa \u003Cbr>Department of Ophthalmology and Visual Sciences, Kyoto University Graduate School of Medicine, Japan |  |  |\n| --- | --- | --- |\n| A R T I C L E I N F O |  | A B S T R A C T |\n| Keywords:\u003Cbr>Primary vitreoretinal lymphoma Intravitreal methotrexate Masquerade syndrome Infiltrative optic neuropathy |  | Purpose: This report presents a diagnostically challenging and aggressive case of primary vitreoretinal lymphoma, demonstrating the potential for recovery following early diagnostic vitrectomy and prompt intravitreal methotrexate.\u003Cbr>Observations: A 42-year-old woman presented with a 1-week history of temporal visual field loss in the right eye and was referred to our hospital for suspected retinal detachment. At presentation, best-corrected visual acuity was 20/16 in the right eye. Fundus examination revealed vitreous cells, retinal hemorrhages, retinal vasculitis, and extensive subretinal fluid. Optical coherence tomography showed preretinal hyperreflective deposits with a level-like appearance. Fluorescein angiography demonstrated vascular leakage and peripheral non-perfusion, while Goldmann perimetry revealed a central scotoma. Despite empiric antimicrobials for presumed infectious uveitis, vision rapidly deteriorated to light perception with worsening of the central scotoma. This profound functional decline was associated with marked optic disc swelling and progressive retinal detachment. Contrastenhanced orbital magnetic resonance imaging demonstrated subtle retrobulbar enhancement, supporting infiltrative optic neuropathy consistent with the central dysfunction. Diagnostic vitrectomy identified a monoclonal B-cell population with an elevated interleukin-10/-6 ratio and clonality of the immunoglobulin heavy-chain gene, confirming primary vitreoretinal lymphoma. Intravitreal methotrexate was initiated the following day. Over eight injections, vision dramatically improved from light perception to 20/20, with resolution of the central scotoma, vitreous opacity, and optic disc swelling.\u003Cbr>Conclusions and importance: This case illustrates a fulminant presentation of primary vitreoretinal lymphoma, characterized by rapidly progressive optic disc involvement and extensive serous retinal detachment. Despite the severity, early diagnostic vitrectomy followed by prompt intravitreal methotrexate enabled anatomical and functional recovery. |\n\n1. Introduction  \nPrimary vitreoretinal lymphoma (PVRL) is a rare but aggressive high-grade malignancy primarily involving the retina, vitreous, or optic nerve head.1–3 Although PVRL may initially present with isolated ocular findings, central nervous system (CNS) involvement eventually developsin 65–90 % of patients.1 The presence of comorbid CNS disease is associated with poor outcomes in PVRL, with a median overall survival of approximately 10 months.4  \nDespite its clinical significance, PVRL is notoriously difficult to diagnose.5 It is a well-known masquerade syndrome, with presentations that mimic infectious retinitis, posterior uveitis, or other systemic  \ninflammatory diseases. Definitive diagnosis requires histopathologic and immunophenotypic confirmation—usually through diagnostic vitrectomy.1 The diagnostic process is invasive and often prolonged, with delays of several months to years reported in large case series, leading to delayed initiation of appropriate therapies. Therefore, accumulating knowledge regarding the ocular manifestations and diagnostic course of PVRL is critically important.  \nHere, w","cbCaiaNnmO5sRosx","https://ap.wps.com/l/cbCaiaNnmO5sRosx","pdf",6076577,"English","# Introduction\n# Case presentation","[{\"question\":\"What condition was ultimately diagnosed in the report?\",\"answer\":\"The final diagnosis was primary vitreoretinal lymphoma (PVRL), confirmed by diagnostic vitrectomy with cytopathology, flow cytometry, cytokine analysis, and molecular studies showing clonality.\"},{\"question\":\"How did the patient’s vision change after treatment?\",\"answer\":\"After prompt intravitreal methotrexate, vision improved dramatically from light perception to 20/20, along with resolution of the central scotoma, vitreous opacity, and optic disc swelling.\"},{\"question\":\"Why was diagnosis difficult in this case?\",\"answer\":\"PVRL presented as a diagnostic mimic resembling infectious retinitis or posterior uveitis, worsening rapidly despite empiric antimicrobials, which delayed definitive identification until invasive diagnostic vitrectomy.\"}]","Rapidly progressive primary vitreoretinal lymphoma with optic disc involvement and retinal detachment | PDF",1790658498,15]