{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T15:24:41Z","timestamp":1771255481340,"version":"3.50.1"},"reference-count":30,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T00:00:00Z","timestamp":1761523200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Primary extranodal marginal zone B-cell lymphoma (EMZL) of the choroid is a rare condition with a handful of cases reported in the literature. Here, we report a challenging case of primary EMZL of the choroid with extrascleral extension and bone marrow involvement. An 86-year-old male presented with 2\u202fyears of progressive vision loss in his left eye. Fundus examination revealed yellow-white subretinal infiltrates at the posterior pole. Ultrasonography detected diffuse choroidal thickening with retrobulbar hypoechoic mass. One year later, an inferior exudative retinal detachment developed. Ultrasonography revealed enlargement of the choroidal low-reflective mass. Another half a year later, visual acuity of the left eye deteriorated to no light perception (NLP) and neovascular glaucoma with intractable pain necessitated enucleation; therefore, enucleation was performed. Histopathological and immunohistochemistry findings confirmed EMZL infiltrates in the choroid and extrascleral tissue, without optic nerve involvement. Concurrent hematologic evaluation revealed significant anemia. The patient was transferred to the hematology department. Bone marrow biopsy demonstrated EMZL involvement morphologically and immunophenotypically identical to the choroidal tumor. Positron emission tomography-computed tomography (PET-CT) revealed no evidence of metabolically active lesions in the lymph nodes, spleen, or visceral organs to suggest an alternate primary tumor. Thus, a diagnosis of primary choroidal EMZL with extrascleral extension and bone marrow involvement was established. To our knowledge, this represents the first reported case of primary choroidal EMZL with bone marrow involvement.<\/jats:p>","DOI":"10.3389\/fmed.2025.1638453","type":"journal-article","created":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T06:20:53Z","timestamp":1761546053000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary choroidal lymphoma with extrascleral extension and bone marrow involvement: a case report"],"prefix":"10.3389","volume":"12","author":[{"given":"Lu","family":"Zhao","sequence":"first","affiliation":[{"name":"Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Department of Ophthalmology, Peking University Third Hospital","place":["Beijing, China"]},{"name":"Beijing Ophthalmic and Visual Science Key Laboratory, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yingyu","family":"Li","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Department of Ophthalmology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pei","family":"Zhang","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Department of Ophthalmology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Changguan","family":"Wang","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Department of Ophthalmology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chunyuan","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology, Lymphoma Center, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huijin","family":"Chen","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Department of Ophthalmology, Peking University Third Hospital","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,27]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1007\/s10384-023-00997-6","article-title":"Diagnosis and management of vitreoretinal lymphoma: present and future treatment perspectives","volume":"67","author":"Kaburaki","year":"2023","journal-title":"Jpn J Ophthalmol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"564","DOI":"10.1111\/j.1442-9071.2008.01843.x","article-title":"Understanding intraocular lymphomas","volume":"36","author":"Coupland","year":"2008","journal-title":"Clin Exp Ophthalmol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1186\/s12886-015-0038-7","article-title":"Primary extranodal marginal zone B-cell lymphoma with diffuse uveal involvement and focal infiltration of the trabecular meshwork: a case report and review of literature","volume":"15","author":"Gao","year":"2015","journal-title":"BMC Ophthalmol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"482","DOI":"10.1007\/s00417-004-1050-4","article-title":"Diagnosis of a primary uveal extranodal marginal zone B-cell lymphoma by chorioretinal biopsy: case report","volume":"243","author":"Coupland","year":"2005","journal-title":"Graefe\u2019s Arch Clin Exp Ophthalmol = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/s0161-6420(99)00025-1","article-title":"Re-evaluation of \u201creactive lymphoid hyperplasia of the uvea\u201d: an Immunohistochemical and molecular analysis of 10 cases","volume":"107","author":"Cockerham","year":"2000","journal-title":"Ophthalmology"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"153","DOI":"10.3322\/caac.21330","article-title":"Clinicopathologic characteristics and treatment of marginal zone Lymphoma of mucosa-associated lymphoid tissue (malt lymphoma)","volume":"66","author":"Raderer","year":"2016","journal-title":"CA Cancer J Clin"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1016\/j.ophtha.2017.05.037","article-title":"Treatments for ocular adnexal lymphoma: a report by the American Academy of ophthalmology","volume":"125","author":"Yen","year":"2018","journal-title":"Ophthalmology"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1159\/000499845","article-title":"Understanding and classification of ocular lymphomas","volume":"5","author":"White","year":"2019","journal-title":"Ocul Oncol Pathol"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"5782","DOI":"10.3390\/cancers15245782","article-title":"Radiotherapy of orbital and ocular adnexa lymphoma: literature review and University of Catania experience","volume":"15","author":"La Rocca","year":"2023","journal-title":"Cancers (Basel)"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"901","DOI":"10.1007\/s00417-004-0973-0","article-title":"Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features","volume":"242","author":"Coupland","year":"2004","journal-title":"Graefe\u2019s Arch Clin Exp Ophthalmol = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1007\/s00417-005-0138-9","article-title":"Results of a retrospective multicentre trial","volume":"244","author":"Jahnke","year":"","journal-title":"Graefe\u2019s Arch Clin Exp Ophthalmol = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1016\/j.ophtha.2013.06.046","article-title":"Choroidal lymphoma: clinical features and association with systemic lymphoma","volume":"121","author":"Mashayekhi","year":"2014","journal-title":"Ophthalmology"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1002\/path.1130","article-title":"Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases","volume":"197","author":"Coupland","year":"2002","journal-title":"J Pathol"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"334","DOI":"10.1016\/j.ophtha.2013.09.004","article-title":"Uveal Lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes","volume":"121","author":"Aronow","year":"2014","journal-title":"Ophthalmology"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1182\/asheducation-2001.1.241","article-title":"Malt lymphomas","volume":"2001","author":"Cavalli","year":"2001","journal-title":"Hematol Am Soc Hematol Educ Program"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1002\/hon.2821","article-title":"The tumor microenvironment of lymphomas: insights into the potential role and modes of actions of checkpoint inhibitors","volume":"39","author":"Menter","year":"2021","journal-title":"Hematol Oncol"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"201","DOI":"10.3324\/haematol.11835","article-title":"Dissemination patterns in non-gastric malt lymphoma","volume":"93","author":"de Boer","year":"2008","journal-title":"Haematologica"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"1010","DOI":"10.1007\/s12032-009-9325-6","article-title":"Primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse epibulbar extension and focal infiltration of the optic nerve and meninges, clinically presented as uveitis masquerade syndrome: a case report","volume":"27","author":"Rasi\u0107","year":"2010","journal-title":"Med Oncol"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1016\/s0039-6257(01)00217-x","article-title":"Variations in the presentation of primary intraocular lymphoma: case reports and a review","volume":"45","author":"Gill","year":"2001","journal-title":"Surv Ophthalmol"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"386","DOI":"10.1016\/s0161-6420(00)00499-1","article-title":"Uveitis masquerade syndromes","volume":"108","author":"Rothova","year":"2001","journal-title":"Ophthalmology"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"153","DOI":"10.2147\/imcrj.S129171","article-title":"Choroidal extranodal marginal zone lymphoma diagnosed by full-thickness retinochoroidal biopsy: case report and review of the literature","volume":"10","author":"Dirani","year":"2017","journal-title":"Int Med Case Rep J"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"828","DOI":"10.1038\/eye.2013.74","article-title":"Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases","volume":"27","author":"Loriaut","year":"2013","journal-title":"Eye (London)"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1080\/09273940590909149","article-title":"Endoretinal biopsy in establishing the diagnosis of uveitis: a clinicopathologic report of three cases","volume":"13","author":"Cassoux","year":"2005","journal-title":"Ocul Immunol Inflamm"},{"key":"ref24","first-page":"84","article-title":"The role of choroidal and retinal biopsies in the diagnosis and management of atypical presentations of uveitis","volume":"103","author":"Lim","year":"2005","journal-title":"Trans Am Ophthalmol Soc"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"8835","DOI":"10.3390\/curroncol29110695","article-title":"Value of combined diagnosis for choroidal lymphoma: a case report","volume":"29","author":"Yang","year":"2022","journal-title":"Curr Oncol"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1016\/j.jcjo.2011.12.017","article-title":"Choroidal lymphoma with orbital and optic nerve extension: case and review of literature","volume":"47","author":"Baryla","year":"2012","journal-title":"Can J Ophthalmol = Journal canadien d\u2019ophtalmologie"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"1382","DOI":"10.1016\/s0360-3016(03)00767-3","article-title":"Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes","volume":"57","author":"Fung","year":"2003","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1016\/j.jaapos.2017.10.016","article-title":"Combined intraocular and systemic rituximab for ocular lymphoproliferative disorder with extranodal marginal zone lymphoma-type morphology after heart transplant","volume":"22","author":"Bata","year":"2018","journal-title":"J Am Assoc Pediatric Ophthalmol Strabismus"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"3935","DOI":"10.2147\/cmar.S267258","article-title":"Chemotherapy-free management of follicular and marginal zone lymphoma","volume":"13","author":"Ollila","year":"2021","journal-title":"Cancer Manag Res"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"874","DOI":"10.1111\/bjh.15843","article-title":"Lenalidomide plus rituximab (R2) in previously untreated marginal zone lymphoma: subgroup analysis and long-term follow-up of an open-label phase 2 trial","volume":"185","author":"Becnel","year":"2019","journal-title":"Br J Haematol"}],"updated-by":[{"DOI":"10.3389\/fmed.2026.1794868","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000}}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2025.1638453\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T14:46:47Z","timestamp":1771253207000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2025.1638453\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,27]]},"references-count":30,"alternative-id":["10.3389\/fmed.2025.1638453"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2025.1638453","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,27]]},"article-number":"1638453"}}