Follicular dendritic cell sarcoma (FDCS) can be divided into the conventional type, and the inflammatory pseudotumor (IPT)-like variant type. Epstein-Barr virus (EBV) infection is considered to be closely associated with the pathogenesis of IPT-like variant of FDCS. Hepatic FDCS has an exceedingly low incidence of only 29 cases reported, with most of these tumors being classified as the IPT-like type. We report a case of an IPT-like variant of FDCS of the liver in a 61-year old man who presented with no marked symptoms. The patient underwent laparoscopic surgery for the mass and was well during a 13-month follow-up periods. The postoperative pathological examination found a proliferation of spindle cells and a diffuse infiltration of inflammatory cells within the tumor. Immunohistochemistry revealed that neoplastic cells were positive for CD23, clusterin, fascin, and PD-L1, and weakly positive for CD35, SMA, and D2-40. The infiltrating lymphocytes were strongly positive for PD1, and IgG4-positive plasma cells were less than 10 cells/high-power field. In situ hybridization for Epstein-Barr virus encoded RNA (EBER) was negative. To our knowledge, the present case is the second case of hepatic IPT-like variant of FDCS without EBV involvement, indicating that EBV infection is not an absolute prerequisite for a diagnosis of the IPT-like variant of FDCS.
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Article Synopsis
- Follicular dendritic cell sarcoma (FDCS) is a rare cancer type, with a specific variant called inflammatory pseudotumor-like FDCS (IPT-like FDCS) having very few established treatment protocols due to its low incidence.
- A case study presented here involved a patient with EBV-positive IPT-like FDCS, initially presenting with thrombocytopenia, for which splenectomy was performed, leading to no signs of recurrence during a one-year follow-up.
- Analysis of 30 cases revealed a slight female predominance, a median age of 62, diverse symptoms, and consistent findings of EBV positivity, with surgical treatment being the common approach for all patients.
Aggressive Variant of Hepatic Epstein-Barr Virus-Associated Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma with PD-L1 and SSTR2a Expression.
Diagnostics (Basel)
September 2023
Rela Institute & Medical Centre, Chennai 6000044, India.
An aggressive Epstein-Barr virus (EBV)-associated inflammatory pseudotumor-like follicular dendritic cell (IPT-like FDC) sarcoma is reported in an adult female. The patient developed multifocal recurrence and passed away 13 months after the initial surgical resection. A bright field microscopic examination of the tumor demonstrated a classical growth pattern and the diffuse expression of Programmed death ligand 1 (PD-L1) and somatostatin receptor 2a (SSTR2a).
View Article and Find Full Text PDFEpstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma Presenting as a Colonic Polyp: Report of a Case with a Literature Review.
Medicina (Kaunas)
July 2023
Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
: Follicular dendritic cell (FDC) sarcoma is an uncommon mesenchymal origin neoplasm derived from the abnormal proliferation and differentiation of FDCs. Epstein‒Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ iFDCS), which used to be known as the inflammatory pseudotumour (IPT)-like variant, occurs exclusively in the liver and spleen and has rarely been reported in the gastrointestinal tract. : Here, we report a case of a 52-year-old woman with a special family history undergoing a routine physical examination.
View Article and Find Full Text PDFInflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma: focus on immunohistochemical profile and association with Epstein-Barr virus.
Infect Agent Cancer
December 2022
Pathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania "Luigi Vanvitelli", Via Luciano Armanni 5, 80100, Naples, Italy.
Inflammatory pseudotumour-like follicular/fibroblastic dendritic cell sarcoma (IPT-like FDCS) is a rare EBV-associated variant of follicular dendritic cell sarcoma, usually arising in the liver or spleen and characterized by a favourable prognosis. The neoplastic cells show variable follicular dendritic cell or fibroblastic reticular cell differentiation and their immunoprofile is still poorly characterized. We describe a case of splenic IPT-like FDCS with unexpected CD31 expression and provide a concise review of English literature on the topic.
View Article and Find Full Text PDFCase report: Hepatic inflammatory pseudotumor-like follicular dendritic cell sarcoma: A rare case and minireview of the literature.
Front Med (Lausanne)
November 2022
Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Inflammatory pseudotumor (IPT)-like follicular dendritic cell sarcoma (FDCS) is a rare neoplasm referred to as the FDCS variant. Here we report a 66-year-old female patient suffering from hepatic IPT-like FDCS and summarize IPT-like FDCS reported in the literature. The patient presented with obvious abdominal pain without significant laboratory abnormalities and subsequently underwent surgical resection of a hepatic lesion.
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