Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature

Abstract Background Follicular dendritic cell sarcoma is a rare stromal tumor with no standard treatment. However, some reports have revealed that follicular dendritic cell sarcoma has an inflammatory pseudotumor variant associated with Epstein–Barr virus infection that has a relatively good prognos...

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Main Authors: K. Abe, M. Kitago, S. Matsuda, M. Shinoda, H. Yagi, Y. Abe, G. Oshima, S. Hori, Y. Endo, T. Yokose, E. Miura, N. Kubota, A. Ueno, Y. Masugi, H. Ojima, M. Sakamoto, Y. Kitagawa
Format: Article
Language:English
Published: Japan Surgical Society 2022-12-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-022-01572-w
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author K. Abe
M. Kitago
S. Matsuda
M. Shinoda
H. Yagi
Y. Abe
G. Oshima
S. Hori
Y. Endo
T. Yokose
E. Miura
N. Kubota
A. Ueno
Y. Masugi
H. Ojima
M. Sakamoto
Y. Kitagawa
author_facet K. Abe
M. Kitago
S. Matsuda
M. Shinoda
H. Yagi
Y. Abe
G. Oshima
S. Hori
Y. Endo
T. Yokose
E. Miura
N. Kubota
A. Ueno
Y. Masugi
H. Ojima
M. Sakamoto
Y. Kitagawa
author_sort K. Abe
collection DOAJ
description Abstract Background Follicular dendritic cell sarcoma is a rare stromal tumor with no standard treatment. However, some reports have revealed that follicular dendritic cell sarcoma has an inflammatory pseudotumor variant associated with Epstein–Barr virus infection that has a relatively good prognosis. In this report, we present a case of a resected inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver, and have reviewed the literature on the clinicopathological, molecular, and genomic features of this tumor. Case presentation The inflammatory pseudotumor variant of follicular dendritic cell sarcoma originates only in the liver or spleen, causes no symptoms, and is more common in middle-aged Asian women. It has no characteristic imaging features, which partially explains why the inflammatory pseudotumor variant of follicular dendritic cell sarcoma is difficult to diagnose. Pathologically, the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has spindle cells mixed with inflammatory cells and is variably positive for follicular dendritic cell markers (CD21, CD23, and CD35) and Epstein–Barr virus-encoded RNA. On genetic analysis, patients with this tumor high levels of latent membrane protein 1 gene expression and extremely low levels of host C–X–C Chemokine Receptor type 7 gene expression, indicating that the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has a latent Epstein–Barr virus type 2 infection. Conclusions The inflammatory pseudotumor variant of follicular dendritic cell sarcoma is an Epstein–Barr virus-associated tumor and a favorable prognosis by surgical resection, similar to Epstein–Barr virus-associated gastric cancer.
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spelling doaj-art-34d08cbb59984effaa1d26baafc5b7322025-08-20T03:28:29ZengJapan Surgical SocietySurgical Case Reports2198-77932022-12-018111010.1186/s40792-022-01572-wEpstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literatureK. Abe0M. Kitago1S. Matsuda2M. Shinoda3H. Yagi4Y. Abe5G. Oshima6S. Hori7Y. Endo8T. Yokose9E. Miura10N. Kubota11A. Ueno12Y. Masugi13H. Ojima14M. Sakamoto15Y. Kitagawa16Department of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Pathology, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineAbstract Background Follicular dendritic cell sarcoma is a rare stromal tumor with no standard treatment. However, some reports have revealed that follicular dendritic cell sarcoma has an inflammatory pseudotumor variant associated with Epstein–Barr virus infection that has a relatively good prognosis. In this report, we present a case of a resected inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver, and have reviewed the literature on the clinicopathological, molecular, and genomic features of this tumor. Case presentation The inflammatory pseudotumor variant of follicular dendritic cell sarcoma originates only in the liver or spleen, causes no symptoms, and is more common in middle-aged Asian women. It has no characteristic imaging features, which partially explains why the inflammatory pseudotumor variant of follicular dendritic cell sarcoma is difficult to diagnose. Pathologically, the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has spindle cells mixed with inflammatory cells and is variably positive for follicular dendritic cell markers (CD21, CD23, and CD35) and Epstein–Barr virus-encoded RNA. On genetic analysis, patients with this tumor high levels of latent membrane protein 1 gene expression and extremely low levels of host C–X–C Chemokine Receptor type 7 gene expression, indicating that the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has a latent Epstein–Barr virus type 2 infection. Conclusions The inflammatory pseudotumor variant of follicular dendritic cell sarcoma is an Epstein–Barr virus-associated tumor and a favorable prognosis by surgical resection, similar to Epstein–Barr virus-associated gastric cancer.https://doi.org/10.1186/s40792-022-01572-wFollicular dendritic cell sarcomaInflammatory pseudotumor variantEpstein–Barr virus-associated tumorClinicopathological featuresMolecular mechanismsLaparoscopic surgery
spellingShingle K. Abe
M. Kitago
S. Matsuda
M. Shinoda
H. Yagi
Y. Abe
G. Oshima
S. Hori
Y. Endo
T. Yokose
E. Miura
N. Kubota
A. Ueno
Y. Masugi
H. Ojima
M. Sakamoto
Y. Kitagawa
Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
Surgical Case Reports
Follicular dendritic cell sarcoma
Inflammatory pseudotumor variant
Epstein–Barr virus-associated tumor
Clinicopathological features
Molecular mechanisms
Laparoscopic surgery
title Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
title_full Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
title_fullStr Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
title_full_unstemmed Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
title_short Epstein–Barr virus-associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver: a case report and review of the literature
title_sort epstein barr virus associated inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver a case report and review of the literature
topic Follicular dendritic cell sarcoma
Inflammatory pseudotumor variant
Epstein–Barr virus-associated tumor
Clinicopathological features
Molecular mechanisms
Laparoscopic surgery
url https://doi.org/10.1186/s40792-022-01572-w
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