{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:40:05Z","timestamp":1786650005725,"version":"build-2736575974"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T00:00:00Z","timestamp":1766966400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose:<\/jats:title>\n                    <jats:p>To describe the clinicopathological characteristics of ophthalmic Rosai\u2013Dorfman disease (RDD).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>A 15-year single-center retrospective case series on ophthalmic RDD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Thirteen cases of ophthalmic RDD were identified, including eight males and five females, with a mean age of 49.2 years. The most common presenting signs were eye redness (76.9%) and proptosis (61.5%). The orbit was the most frequently involved site (84.6%). Epibulbar masses occurred in 53.8% of cases, and one case had a concurrent uveal lesion. Eyelid involvement was noted in 46.2% (six cases). Compressive optic neuropathy was documented in 23.1% (three patients). Bone erosion was observed in 53.8% (seven patients). Systemic involvement was present in 69.2% (9 patients), and lymphadenopathy was detected in 15.4% (two patients). Paranasal sinus infiltration occurred in 38.5% (five patients). Other systemic associations included skin and retroperitoneum\/kidney (both 15.4%), cerebrum (7.7%), and cardiovascular system (7.7%). Histopathology revealed large S-100-positive histiocytes with emperipolesis in an inflammatory and fibrotic background in all cases. All were BRAF V600E negative, except one with a coexisting Erdheim\u2013Chester-like lesion, suggesting mixed histiocytosis due to overlapping phenotypes with Erdheim\u2013Chester disease (ECD). Increased IgG4-positive plasma cells were seen in 38.5% (five cases).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Ophthalmic RDD most frequently presents with orbital and epibulbar masses and rarely involves the uvea. Increased IgG4-positive plasma cells in some RDD cases necessitate careful differentiation from IgG4-related disease. In rare conditions, overlapping clinicopathological phenotypes with ECD and BRAF V600E mutation suggest a diagnosis of mixed RDD\/ECD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/ijo.ijo_902_25","type":"journal-article","created":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T15:00:14Z","timestamp":1767020414000},"page":"111-116","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Clinicopathological insights into ophthalmic Rosai\u2013Dorfman disease: A 15-year retrospective analysis"],"prefix":"10.4103","volume":"74","author":[{"given":"Rongrong","family":"Cai","sequence":"first","affiliation":[{"name":"Department of Pathology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingwen","family":"Bi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongqin","family":"Jia","sequence":"additional","affiliation":[{"name":"Department of Pathology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yifei","family":"Yuan","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pathology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiahao","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Eye and ENT Hospital of Fudan University, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,12,29]]},"reference":[{"key":"R1-20260420","doi-asserted-by":"crossref","first-page":"2877","DOI":"10.1182\/blood-2018-03-839753","article-title":"Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease","volume":"131","author":"Abla","year":"2018","journal-title":"Blood"},{"key":"R2-20260420","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1038\/s41375-022-01613-1","article-title":"The 5th edition of the World Health organization classification of haematolymphoid tumours: Myeloid and histiocytic\/dendritic neoplasms","volume":"36","author":"Khoury","year":"2022","journal-title":"Leukemia"},{"key":"R3-20260420","doi-asserted-by":"crossref","first-page":"348","DOI":"10.3324\/haematol.2019.219626","article-title":"Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease","volume":"105","author":"Goyal","year":"2020","journal-title":"Haematologica"},{"key":"R4-20260420","doi-asserted-by":"crossref","first-page":"2297","DOI":"10.1038\/s41375-023-02032-6","article-title":"Clinical phenotypes, molecular analysis, and outcomes of patients with Rosai-Dorfman disease","volume":"37","author":"Chang","year":"2023","journal-title":"Leukemia"},{"key":"R5-20260420","doi-asserted-by":"crossref","first-page":"40","DOI":"10.5858\/arpa.2021-0116-RA","article-title":"Rosai-Dorfman disease of bone and soft tissue","volume":"146","author":"Garcia","year":"2022","journal-title":"Arch Pathol Lab Med"},{"key":"R6-20260420","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1186\/s12886-021-02173-1","article-title":"Ophthalmic Rosai-Dorfman disease: A multi-centre comprehensive study","volume":"21","author":"Alzahem","year":"2021","journal-title":"BMC Ophthalmol"},{"key":"R7-20260420","doi-asserted-by":"crossref","first-page":"1216","DOI":"10.1177\/11206721221138319","article-title":"Rosai-Dorfman Disease involving the eye and ocular adnexa. Clinicopathologic series of 17 patients","volume":"33","author":"Rios","year":"2022","journal-title":"Eur J Ophthalmol"},{"key":"R8-20260420","doi-asserted-by":"crossref","first-page":"e18757","DOI":"10.1097\/MD.0000000000018757","article-title":"Rosai-Dorfman disease manifesting as epibulbar and orbital tumor: A case report and literature review","volume":"99","author":"Huang","year":"2020","journal-title":"Medicine (Baltimore)"},{"key":"R9-20260420","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1016\/j.ajo.2018.01.037","article-title":"Ophthalmic findings of Rosai-Dorfman disease","volume":"188","author":"Choi","year":"2018","journal-title":"Am J Ophthalmol"},{"key":"R10-20260420","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1097\/01.iop.0000217563.00975.a3","article-title":"The spectrum of orbital Rosai-Dorfman disease","volume":"22","author":"Mohadjer","year":"2006","journal-title":"Ophthalmic Plast Reconstr Surg"},{"key":"R11-20260420","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1136\/jclinpath-2020-206733","article-title":"Rosai-Dorfman disease: An overview","volume":"73","author":"Bruce-Brand","year":"2020","journal-title":"J Clin Pathol"},{"key":"R12-20260420","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.ophtha.2022.07.031","article-title":"Ophthalmologic involvement in adults with histiocytic disorders: Clinical presentation and treatment outcomes","volume":"130","author":"Banks","year":"2023","journal-title":"Ophthalmology"},{"key":"R13-20260420","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1097\/IOP.0000000000000589","article-title":"Extranodal Rosai-Dorfman disease of the orbit: Clinical features of 8 cases","volume":"32","author":"Tan","year":"2016","journal-title":"Ophthalmic Plast Reconstr Surg"},{"key":"R14-20260420","doi-asserted-by":"crossref","first-page":"238","DOI":"10.4103\/JOCO.JOCO_84_20","article-title":"Ophthalmic manifestations of Rosai-Dorfman disease in five patients","volume":"32","author":"Masoomian","year":"2020","journal-title":"J Curr Ophthalmol"},{"key":"R15-20260420","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1080\/09273948.2021.1986547","article-title":"Bilateral ocular rosai-dorfman disease presenting as diffuse anterior scleritis with multiple enlarging epibulbar masses","volume":"31","author":"Agarwal","year":"2023","journal-title":"Ocul Immunol Inflamm"},{"key":"R16-20260420","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1186\/s12886-019-1158-2","article-title":"Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: A case report","volume":"19","author":"Lee","year":"2019","journal-title":"BMC Ophthalmol"},{"key":"R17-20260420","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/j.hemonc.2018.05.005","article-title":"Rosai-Dorfman disease masquerading as Uveal Melanoma: Case report and review of literature","volume":"13","author":"Yousef","year":"2020","journal-title":"Hematol Oncol Stem Cell Ther"},{"key":"R18-20260420","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1159\/000497185","article-title":"Isolated intraocular Rosai-Dorfman disease","volume":"5","author":"Fogt","year":"2019","journal-title":"Ocul Oncol Pathol"},{"key":"R19-20260420","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1186\/s13023-020-01567-6","article-title":"Rosai\u2013Dorfman disease mimicking IgG4-related diseases: A single-center experience in China","volume":"15","author":"Wang","year":"2020","journal-title":"Orp hanet J Rare Dis"},{"key":"R20-20260420","doi-asserted-by":"crossref","first-page":"2672","DOI":"10.1182\/blood-2016-01-690636","article-title":"Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages","volume":"127","author":"Emile","year":"2016","journal-title":"Blood"},{"key":"R21-20260420","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1007\/s10384-024-01072-4","article-title":"The 2023 revised diagnostic criteria for IgG4-related ophthalmic disease","volume":"68","author":"Takahira","year":"2024","journal-title":"Jpn J Ophthalmol"},{"key":"R22-20260420","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1016\/S0140-6736(21)01812-2","article-title":"IgG4-related disease and Rosai-Dorfman-Destombes disease","volume":"398","author":"Chen","year":"2021","journal-title":"Lancet"},{"key":"R23-20260420","doi-asserted-by":"crossref","first-page":"e5","DOI":"10.3324\/haematol.2019.216937","article-title":"Erdheim-Chester disease with concomitant Rosai-Dorfman like lesions: A distinct entity mainly driven by MAP2K1","volume":"105","author":"Razanamahery","year":"2020","journal-title":"Haematologica"},{"key":"R24-20260420","doi-asserted-by":"crossref","first-page":"e014582","DOI":"10.1161\/CIRCIMAGING.122.014582","article-title":"Rosai-Dorfman disease involving the descending aorta","volume":"16","author":"Li","year":"2023","journal-title":"Circ Cardiovasc Imaging"},{"key":"R25-20260420","doi-asserted-by":"crossref","first-page":"e377","DOI":"10.3324\/haematol.2018.190934","article-title":"BRAF V600E mutation detected in a case of Rosai-Dorfman disease","volume":"103","author":"Fatobene","year":"2018","journal-title":"Haematologica"},{"key":"R26-20260420","doi-asserted-by":"crossref","first-page":"1848","DOI":"10.1182\/bloodadvances.2019000093","article-title":"BRAF-V600E-mutated Rosai-Dorfman-Destombes disease and Langerhans cell histiocytosis with response to BRAF inhibitor","volume":"3","author":"Mastropolo","year":"2019","journal-title":"Blood Adv"},{"key":"R27-20260420","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/j.neurol.2020.09.010","article-title":"BRAF mutation in overlapping form of Erdheim-Chester and Rosai Dorfman diseases: A unique case restricted to the central nervous system","volume":"177","author":"Dufour","year":"2021","journal-title":"Rev Neurol (Paris)"},{"key":"R28-20260420","doi-asserted-by":"crossref","first-page":"1070828","DOI":"10.3389\/fmed.2022.1070828","article-title":"Case report: BRAF-inhibitor therapy in BRAF-mutated primary CNS tumours including one case of BRAF-mutated Rosai-Dorfman disease","volume":"9","author":"Cronin","year":"2022","journal-title":"Front Med (Lausanne)"},{"key":"R29-20260420","doi-asserted-by":"crossref","first-page":"3984","DOI":"10.1182\/bloodadvances.2022009013","article-title":"Successful treatment of non-Langerhans cell histiocytosis with the MEK inhibitor trametinib: A multicenter analysis","volume":"7","author":"Aaroe","year":"2023","journal-title":"Blood Adv"},{"key":"R30-20260420","doi-asserted-by":"crossref","first-page":"388","DOI":"10.32074\/1591-951X-548","article-title":"Rosai-Dorfman disease. A legacy of Professor Rosai that is still not exploited completely","volume":"113","author":"Doglioni","year":"2021","journal-title":"Pathologica"},{"key":"R31-20260420","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/ajcp\/aqad047","article-title":"How I diagnose Rosai-Dorfman disease","volume":"160","author":"Ravindran","year":"2023","journal-title":"Am J Clin Pathol"},{"key":"R32-20260420","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1111\/j.1442-9071.2010.02404.x","article-title":"Rosai-Dorfman disease manifesting as an epibulbar ocular tumour","volume":"39","author":"de Oliveira","year":"2011","journal-title":"Clin Exp Ophthalmol"},{"key":"R33-20260420","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1001\/jamaoncol.2022.4432","article-title":"Outcomes after treatment with cobimetinib in patients with Rosai-Dorfman disease based on KRAS and MEK alteration status","volume":"8","author":"Abeykoon","year":"2022","journal-title":"JAMA Oncol"}],"container-title":["Indian Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/IJO.IJO_902_25","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:00:59Z","timestamp":1786647659000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/IJO.IJO_902_25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,29]]},"references-count":33,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/ijo.ijo_902_25","relation":{},"ISSN":["0301-4738","1998-3689"],"issn-type":[{"value":"0301-4738","type":"print"},{"value":"1998-3689","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,29]]},"assertion":[{"value":"2025-04-09","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-09-22","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}