Follicular dendritic cell tumor of the liver: a clinicopathologic and Epstein-Barr virus study of two cases

Mod Pathol. 2001 Apr;14(4):354-60. doi: 10.1038/modpathol.3880315.

Abstract

Two cases of hepatic follicular dendritic cell (FDC) tumor are described. Both patients were female, aged 57 and 51 years. They presented with epigastralgia or abdominal fullness and weight loss. The first patient refused surgical resection. She developed progressive polyclonal gammopathy and then bilateral purpura over the legs. Skin biopsy revealed leukocytoclastic vasculitis with granular vascular deposits of IgA and C3. The second patient had marked peripheral blood and tissue eosinophilia. The histological diagnosis was confirmed by positive staining for CD21 and CD23. The stromal lymphocytes were predominantly composed of CD3(+)and CD8(+) cells. In situ hybridization for EBER showed a positive nuclear signal in tumor cells but not in inflammatory cells. Polymerase chain reaction amplification for Exon 3 of the latent membrane protein-1 (LMP-1) gene showed a characteristic 30-bp deletion between nucleotides 168282 and 168253, corresponding to the B95-8 sequence. The unique clinicopathological features of our cases have not been reported for FDC tumors before. The clinical significance of the 30-bp deletion in Exon 3 of the LMP-1 gene in FDC tumor of the liver warrants further investigation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Complement C3 / metabolism
  • DNA, Neoplasm / analysis
  • Dendritic Cells / chemistry
  • Dendritic Cells / pathology*
  • Dendritic Cells / virology
  • Female
  • Fluorescent Antibody Technique, Direct
  • Gene Deletion
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / isolation & purification
  • Herpesvirus 4, Human / pathogenicity*
  • Humans
  • Immunoglobulin A / metabolism
  • In Situ Hybridization
  • Liver Neoplasms / chemistry
  • Liver Neoplasms / pathology*
  • Liver Neoplasms / virology
  • Lymphoma, Follicular / chemistry
  • Lymphoma, Follicular / pathology*
  • Lymphoma, Follicular / virology
  • Middle Aged
  • Polymerase Chain Reaction
  • RNA, Viral / analysis
  • Receptors, Complement 3d / analysis
  • Receptors, IgE / analysis
  • Skin / metabolism
  • Skin / pathology
  • Vasculitis, Leukocytoclastic, Cutaneous / metabolism
  • Vasculitis, Leukocytoclastic, Cutaneous / pathology
  • Viral Matrix Proteins / analysis
  • Viral Matrix Proteins / genetics

Substances

  • Complement C3
  • DNA, Neoplasm
  • EBV-associated membrane antigen, Epstein-Barr virus
  • Epstein-Barr virus encoded RNA 1
  • Immunoglobulin A
  • RNA, Viral
  • Receptors, Complement 3d
  • Receptors, IgE
  • Viral Matrix Proteins