Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma with significant granuloma : case report and literature review
© 2024. The Author(s)..
BACKGROUND: Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is a rare disease characterized by mild clinical symptoms and non-specific imaging findings. The diagnosis of the disease depends on pathological diagnosis. However, EBV+IFDCS has a very broad spectrum of histological morphology and immune phenotypes, and its histopathological features have not been fully described by pathologists.
CASE PRESENTATION: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case.
CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:19 |
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| Enthalten in: |
Diagnostic pathology - 19(2024), 1 vom: 16. Feb., Seite 34 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Nie, Chenchen [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 19.02.2024 Date Revised 19.02.2024 published: Electronic Citation Status MEDLINE |
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| doi: |
10.1186/s13000-024-01457-6 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM368558029 |
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| 500 | |a Date Revised 19.02.2024 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2024. The Author(s). | ||
| 520 | |a BACKGROUND: Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is a rare disease characterized by mild clinical symptoms and non-specific imaging findings. The diagnosis of the disease depends on pathological diagnosis. However, EBV+IFDCS has a very broad spectrum of histological morphology and immune phenotypes, and its histopathological features have not been fully described by pathologists | ||
| 520 | |a CASE PRESENTATION: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case | ||
| 520 | |a CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases | ||
| 650 | 4 | |a Review | |
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Case report | |
| 650 | 4 | |a Epithelioid granulomas | |
| 650 | 4 | |a Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma | |
| 650 | 4 | |a Follicular dendritic cell sarcoma | |
| 650 | 4 | |a Inflammatory pseudotumor-like follicular dendritic cell sarcoma | |
| 700 | 1 | |a Xie, Xun |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Hangyan |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Yangcan |e verfasserin |4 aut | |
| 700 | 1 | |a Chen, Zhihong |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Yanchun |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Zhenfeng |e verfasserin |4 aut | |
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