{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:47:17Z","timestamp":1792000037371,"version":"4.4.0"},"reference-count":40,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:p>Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell (FDC) sarcoma (EBV<jats:sup>+<\/jats:sup> IFDCS) is a rare entity, and its histopathological characteristics have not been fully described. Here, we investigated the wide morphologic spectrum and immunophenotype of this tumor with the aim to help avoid misdiagnosis. Thirteen cases of EBV<jats:sup>+<\/jats:sup> IFDCS were retrospectively analyzed, combined with a review of 70 cases reported in the literature. The median age of patients was 49 (range, 29 to 67\u00a0y). Six patients were male and 7 were female. Most cases (92.3%, 12\/13) occurred in the liver or spleen, and only 1 case affected an extra-hepatosplenic site (lung, 7.7%, 1\/13). Tumors were assessed for a variety of histologic features and assigned to the following morphologic groups: classic type (53.8%, 7\/13), lymphoma-like subtype (38.5%, 5\/13), and hemangioma-like subtype (7.7%, 1\/13). The classic type had distinct EBV-positive neoplastic cells with a fascicular or storiform growth pattern, variable lymphoplasmacytic infiltrates, and blood vessels. The lymphoma-like subtype had extremely prominent lymphoplasmacytic infiltrates (resembling marginal zone lymphoma with plasmacytoid differentiation) with singly dispersed distinct EBV-positive neoplastic cells, highlighted by in situ hybridization for EBV-encoded small RNA. The hemangioma-like subtype had extremely prominent blood vessels with hyaline and\/or fibrinoid degeneration, singly dispersed distinct EBV-positive neoplastic cells, and limited lymphoplasmacytic infiltrates. Immunohistochemically, the neoplastic cells showed variable staining for FDC markers (CD21, CD35, CD23, and SSTR2) and the fibroblastic marker SMA, with the staining ranging from very focal to extensive. The number of EBV-positive neoplastic cells ranged from 80 to 400\/HPF. All cases showed variable expression of PD-ligand 1 (PD-L1) (CPS: 5-90). IgG4-positive cells ranged from rare up to 100\/HPF. Interestingly, 2 cases satisfied the criteria proposed in a previous study, mimicking IgG4-related disease. EBV<jats:sup>+<\/jats:sup> IFDCS is an entity with an extremely wide morphologic spectrum and immunophenotype. Awareness of the spectrum of morphologic presentations of this rare tumor, specifically the lymphoma-like subtype and hemangioma-like subtype, is important for accurate diagnosis.<\/jats:p>","DOI":"10.1097\/pas.0000000000002011","type":"journal-article","created":{"date-parts":[[2022,12,27]],"date-time":"2022-12-27T18:00:09Z","timestamp":1672164009000},"page":"476-489","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":34,"title":["Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma"],"prefix":"10.1097","volume":"47","author":[{"given":"Yan","family":"Li","sequence":"first","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xia","family":"Yang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lili","family":"Tao","sequence":"additional","affiliation":[{"name":"Department of Pathology, Peking University Shenzhen Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weimei","family":"Zeng","sequence":"additional","affiliation":[{"name":"Guangdong No. 2 Provincial People\u2019s Hospital, Guangzhou, Guangdong"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Zuo","sequence":"additional","affiliation":[{"name":"Department of Pathology, Shenzhen People\u2019s Hospital, Shenzhen, Guangdong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuo","family":"Li","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liyan","family":"Wu","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanshong","family":"Lin","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziying","family":"Zhang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingping","family":"Yun","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuhua","family":"Huang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine"},{"name":"Department of Pathology, Sun Yat-sen University Cancer Center"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,12,28]]},"reference":[{"key":"R1-20250608","first-page":"155","article-title":"Detection of Epstein-Barr Virus in inflammatory pseudotumor","volume":"15","author":"Arber","year":"1998","journal-title":"Semin Diagn Pathol"},{"key":"R2-20250608","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1097\/00000478-200106000-00003","article-title":"Inflammatory pseudotumor-like follicular dendritic cell tumor: a distinctive low-grade malignant intra-abdominal neoplasm with consistent Epstein-Barr virus association","volume":"25","author":"Cheuk","year":"2001","journal-title":"Am J Surg Pathol"},{"key":"R3-20250608","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1097\/00000478-199606000-00013","article-title":"Inflammatory pseudotumor of the liver. 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