Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma Presenting as a Colonic Polyp: Report of a Case with a Literature Review

Medicina (Kaunas). 2023 Jul 21;59(7):1341. doi: 10.3390/medicina59071341.

Abstract

Background: 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. Case study: Here, we report a case of a 52-year-old woman with a special family history undergoing a routine physical examination. The colonoscope revealed an approximately 18 mm transverse colonic polyp, and the endoscopic polypectomy was performed. Microscopically, the excised polypoid mass was composed predominantly of inflammatory cells scattered with atypical ovoid to spindle tumor cells. Interestingly, there was a remarkable infiltration of IgG4+ cells. Immunohistochemistry showed that the tumor cells were positive for CD21, CD23 and CD35. EBV-encoded mRNA (EBER) in situ hybridization also gave positive signals. These histopathology features supported the diagnosis of EBV+ iFDCS. The patient was free of disease over 1-year follow-up. Conclusion: Identification of the potential pathogenesis sites of EBV+ iFDCS in extra-hepatosplenic regions is necessary for correct and timely diagnosis, and we consider it very meaningful to share our experience of diagnosing this tumor type. Furthermore, we summarize the clinicopathological features of EBV+ iFDCS presenting as a colon polyp after a thorough review of the literature.

Keywords: Epstein-Barr virus; colon polyp; follicular dendritic cell sarcoma; inflammatory; lgG4.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Colonic Polyps* / diagnosis
  • Dendritic Cell Sarcoma, Follicular* / diagnosis
  • Dendritic Cell Sarcoma, Follicular* / metabolism
  • Dendritic Cell Sarcoma, Follicular* / pathology
  • Epstein-Barr Virus Infections* / complications
  • Epstein-Barr Virus Infections* / diagnosis
  • Female
  • Herpesvirus 4, Human / genetics
  • Humans
  • Liver / pathology
  • Middle Aged

Grants and funding

This article was supported by the Postgraduate Education Society of Zhejiang province (Num.2019-021) and the Education and Teaching Reform Project in the First Affiliated Hospital, Zhejiang University School of Medicine (Num.zyjg202013).