{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:28:14Z","timestamp":1790393294938,"version":"4.1.0"},"reference-count":25,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,4,25]],"date-time":"2024-04-25T00:00:00Z","timestamp":1714003200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Ocular adnexa region (OAR) primary lymphomas are uncommon, accounting for 1\u20132% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Extranodal marginal zone lymphoma (EMZL) originates from several epithelial tissues, including the stomach, salivary gland, lung, small intestine, thyroid gland, and ocular adnexa region. Here, we report a 66-year-old female patient who was diagnosed with EMZL of OAR. In consideration of the possible side effect of radiotherapy, such as conjunctivitis, visual acuity impairment, and even retinal complications, she received six cycles of triweekly targeted chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) without radiotherapy. Then, she remained in complete remission up to the present day.<\/jats:p>","DOI":"10.3390\/medicina60050706","type":"journal-article","created":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T09:26:02Z","timestamp":1714123562000},"page":"706","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Rare Orbital Involvement Originating from Extranodal Marginal Zone Lymphoma"],"prefix":"10.3390","volume":"60","author":[{"given":"Yao-Chang","family":"Wen","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei 114202, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tzu-Chuan","family":"Huang","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei 114202, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen-Chiuan","family":"Tsai","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei 114202, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2928-5401","authenticated-orcid":false,"given":"Shiue-Wei","family":"Lai","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei 114202, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1002\/1097-0142(197201)29:1<252::AID-CNCR2820290138>3.0.CO;2-#","article-title":"Occurrence and prognosis of extranodal lymphoma","volume":"29","author":"Freeman","year":"1972","journal-title":"Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1097\/01.pas.0000213350.49767.46","article-title":"Lymphoma of the ocular adnexa: A study of 353 cases","volume":"31","author":"Ferry","year":"2007","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Kaddu-Mulindwa, D., Thurner, L., Christofyllakis, K., Bewarder, M., and Kos, I.A. (2022). Management of Extranodal Marginal Zone Lymphoma: Present and Upcoming Perspectives. Cancers, 14.","DOI":"10.3390\/cancers14123019"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1093\/annonc\/mdm513","article-title":"OAML: An intriguing model for antigen-driven lymphomagenesis and microbial targeted therapy","volume":"19","author":"Ferreri","year":"2008","journal-title":"Ann. Oncol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1177\/107327481602300208","article-title":"Extranodal Marginal Zone B-cell Lymphoma of the Ocular Adnexa","volume":"23","author":"Johnson","year":"2016","journal-title":"Cancer Control."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1002\/1097-0142(19930801)72:3<843::AID-CNCR2820720333>3.0.CO;2-#","article-title":"The clinical spectrum of ocular lymphoma","volume":"72","author":"Peterson","year":"1993","journal-title":"Cancer"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"22","DOI":"10.3816\/CLM.2003.n.010","article-title":"Primary ocular lymphoma: Clinical features, diagnosis, and treatment","volume":"4","author":"Hormigo","year":"2003","journal-title":"Clin. Lymphoma"},{"key":"ref_8","unstructured":"(2023, October 19). NCCN Clinical Practice Guidelines in Oncology, EMZLMG-2, ver. 6.2023\u201410 October 2023. Available online: https:\/\/jnccn.org\/view\/journals\/jnccn\/21\/11\/article-p1118.xml."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1002\/cncr.11539","article-title":"Radiotherapy for extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: A multi-institutional, retrospective review of 50 patients","volume":"98","author":"Uno","year":"2003","journal-title":"Cancer"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.clon.2014.10.002","article-title":"Outcome of primary lymphoma of the ocular adnexal (orbital lymphoma) treated with radiotherapy","volume":"27","author":"Woolf","year":"2015","journal-title":"Clin. Oncol. R Coll. Radiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1509","DOI":"10.1016\/j.ijrobp.2011.04.052","article-title":"Long-term outcome and patterns of failure of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma treated with radiotherapy","volume":"82","author":"Hashimoto","year":"2012","journal-title":"Int. J. Radiat. Oncol. Biol. Phys."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Kiesewetter, B., Lukas, J., Kuchar, A., Mayerhoefer, M.E., Streubel, B., Lagler, H., M\u00fcllauer, L., W\u00f6hrer, S., Fischbach, J., and Raderer, M. (2014). Clinical features, treatment and outcome of mucosa-associated lymphoid tissue (MALT) lymphoma of the ocular adnexa: Single center experience of 60 patients. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0104004"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3153","DOI":"10.2147\/OPTH.S263291","article-title":"Ocular Complications After Radiation Therapy: An Observational Study","volume":"14","author":"Nuzzi","year":"2020","journal-title":"Clin. Ophthalmol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1016\/j.ijrobp.2013.04.035","article-title":"Low-Dose Radiation Therapy (2 Gy \u00d7 2) in the Treatment of Orbital Lymphoma","volume":"86","author":"Fasola","year":"2013","journal-title":"Int. J. Radiat. Oncol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"780","DOI":"10.3109\/02713683.2014.959605","article-title":"Systemic rituximab immunotherapy in the management of primary ocular adnexal lymphoma: Single institution experience","volume":"40","author":"Tuncer","year":"2015","journal-title":"Curr. Eye Res."},{"key":"ref_16","first-page":"1578","article-title":"Rituximab in patients with mucosa-associated lymphoid tissue type lymphoma of the ocular adnexa","volume":"90","author":"Ferreri","year":"2005","journal-title":"Haematologica"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1093\/annonc\/mdm457","article-title":"Efficacy of chemotherapy as a first-line treatment in ocular adnexal extranodal marginal zone B-cell lymphoma","volume":"19","author":"Song","year":"2008","journal-title":"Ann. Oncol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1016\/j.ophtha.2006.01.057","article-title":"Oral chlorambucil for extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the orbit","volume":"113","author":"Cheung","year":"2006","journal-title":"Ophthalmology"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1182\/blood-2006-02-005017","article-title":"Ocular adnexal lymphoma: A review of clinicopathologic features and treatment options","volume":"108","author":"Decaudin","year":"2006","journal-title":"Blood"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1007\/s00277-011-1337-6","article-title":"Phase II trial of rituximab plus CVP combination chemotherapy for advanced stage marginal zone lymphoma as a first-line therapy: Consortium for Improving Survival of Lymphoma (CISL) study","volume":"91","author":"Kang","year":"2012","journal-title":"Ann. Hematol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1002\/hon.2105","article-title":"Treatment of gastric marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue with rituximab, cyclophosphamide, vincristine and prednisone","volume":"32","year":"2014","journal-title":"Hematol. Oncol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s40880-019-0403-7","article-title":"Phase II study of R-CVP followed by rituximab maintenance therapy for patients with advanced marginal zone lymphoma: Consortium for improving survival of lymphoma (CISL) study","volume":"39","author":"Oh","year":"2019","journal-title":"Cancer Commun."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2944","DOI":"10.1182\/blood-2013-11-531327","article-title":"Randomized trial of bendamustine-rituximab or R-CHOP\/R-CVP in first-line treatment of indolent NHL or MCL: The BRIGHT study","volume":"123","author":"Flinn","year":"2014","journal-title":"Blood"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1817","DOI":"10.1016\/S0140-6736(13)60313-X","article-title":"Rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone in patients with newly diagnosed diffuse large B-cell non-Hodgkin lymphoma: A phase 3 comparison of dose intensification with 14-day versus 21-day cycles","volume":"381","author":"Cunningham","year":"2013","journal-title":"Lancet"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.annonc.2019.10.010","article-title":"Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann","volume":"31","author":"Zucca","year":"2020","journal-title":"Oncol. Off. J. Eur. Soc. Med. Oncol."}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/5\/706\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:33:57Z","timestamp":1760106837000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/5\/706"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,25]]},"references-count":25,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2024,5]]}},"alternative-id":["medicina60050706"],"URL":"https:\/\/doi.org\/10.3390\/medicina60050706","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,25]]}}}