{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T12:50:38Z","timestamp":1782478238586,"version":"3.54.5"},"reference-count":30,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T00:00:00Z","timestamp":1780012800000},"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. Immunol."],"abstract":"<jats:p>Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor involving lymph nodes and extranodal sites, classified into conventional and inflammatory pseudotumor (IPT)-like variants. The IPT-like variant predominantly involves the liver and spleen. Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disorder characterized by multiorgan involvement, whereas isolated esophageal and gastric involvement is exceedingly rare. Although FDCS may exhibit infiltration of IgG4-positive plasma cells, the coexistence of FDCS and IgG4-RD has not been previously reported. Herein, we describe a unique case of FDCS associated with IgG4-RD involving the liver, esophagus, and stomach. The patient initially presented with hepatic lesions and gastrointestinal ulcers, accompanied by elevated serum IgG4 levels and histopathological features suggestive of IgG4-RD. Following glucocorticoid and immunosuppressive therapy, gastrointestinal lesions improved; however, the hepatic lesion progressed and was subsequently confirmed as FDCS by histopathology and immunohistochemistry. This case highlights the diagnostic challenges posed by overlapping clinicopathological features between FDCS and IgG4-RD.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1685017","type":"journal-article","created":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T06:11:29Z","timestamp":1780035089000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Follicular dendritic cell sarcoma associated with IgG4-related disease: a case report and literature review"],"prefix":"10.3389","volume":"17","author":[{"given":"Yajuan","family":"Yao","sequence":"first","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunjing","family":"Bai","sequence":"additional","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guang","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Du","sequence":"additional","affiliation":[{"name":"Department of Pathology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyu","family":"Long","sequence":"additional","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaohua","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fan","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaorong","family":"Jin","sequence":"additional","affiliation":[{"name":"Department of Rheumatism and Immunology, Seventh Medical Center of Chinese PLA General Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,29]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.32074\/1591-951X-331","article-title":"Follicular dendritic cell sarcoma","volume":"113","author":"Facchetti","year":"2021","journal-title":"Pathologica"},{"key":"B2","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1002\/art.41120","article-title":"The 2019 American college of rheumatology\/European league against rheumatism classification criteria for igG4-related disease","volume":"72","author":"Wallace","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.53.179","article-title":"Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis","volume":"53","author":"Ohtake","year":"2013","journal-title":"J Clin Exp Hematop"},{"key":"B4","article-title":"A primary lymph node Malignancy with features suggestive of dendritic reticulum cell differentiation. 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