Inflammatory Pseudotumor-Like Follicular/Fibroblastic Dendritic Cell Sarcomas of the Spleen Are EBV-Associated and Lack Other Commonly Identifiable Molecular Alterations

Int J Surg Pathol. 2021 Jun;29(4):443-446. doi: 10.1177/1066896920949675. Epub 2020 Aug 12.

Abstract

Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma (IPT-like FFDCS) is a rare, indolent neoplasm that occurs in the spleen or liver and harbors Epstein-Barr virus (EBV) integrated into the host genome. The molecular genetic characteristics of IPT-like FFDCS have not been well studied and there are no established and actionable molecular features to guide treatment decisions or diagnosis beyond the recognition of viral genome integration. We subjected two cases of IPT-like FFDCS to a comprehensive next-generation sequencing analysis. Several variants of uncertain clinical significance were detected in both tumors. No variants of potential or strong clinical significance were detected within the targeted regions of the evaluated genes. Additionally, no fusion events were detected involving the genes in either tumor. The performed molecular analysis identified no genetic aberrations in IPT-like FFDCS and its genomic landscape remains, with the exception of a monoclonal EBV gene, largely undefined.

Keywords: BRAF; Epstein-Barr virus; NGS; inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma; spleen.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Biomarkers, Tumor / genetics
  • DNA Mutational Analysis
  • DNA, Viral / isolation & purification
  • Dendritic Cell Sarcoma, Follicular / diagnosis*
  • Dendritic Cell Sarcoma, Follicular / genetics
  • Dendritic Cell Sarcoma, Follicular / surgery
  • Dendritic Cell Sarcoma, Follicular / virology
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / genetics
  • Epstein-Barr Virus Infections / surgery
  • Epstein-Barr Virus Infections / virology
  • Female
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / isolation & purification*
  • Humans
  • Male
  • Spleen / diagnostic imaging
  • Spleen / pathology*
  • Spleen / surgery
  • Spleen / virology
  • Splenectomy
  • Splenic Neoplasms / diagnosis*
  • Splenic Neoplasms / genetics
  • Splenic Neoplasms / surgery
  • Splenic Neoplasms / virology
  • Tomography, X-Ray Computed

Substances

  • Biomarkers, Tumor
  • DNA, Viral