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Inflammatory Pseudotumor of the Spleen Associated With a Clonal Epstein-Barr Virus Genome
Case Report and Review of the Literature

Jason T. Lewis MD, Robyn L. Gaffney MD, Mary B. Casey MD, Michael A. Farrell MD, William G. Morice MD, PhD, William R. Macon MD
DOI: http://dx.doi.org/10.1309/BUWNMG5RV4D09YYH 56-61 First published online: 1 July 2003


We report a case of an inflammatory pseudotumor (IPT) of the spleen occurring in an 81-year-old woman with a history of a monoclonal gammopathy of undetermined significance. Eighteen-month follow-up after splenectomy demonstrated no tumor recurrence or progression of underlying plasma cell disease. Histologic examination of the tumor demonstrated a polymorphic population of inflammatory and epithelioid and spindle cells. Immunophenotyping showed large numbers of T cells, B cells, and polyclonal plasma cells. The epithelioid and spindle cells were positive for vimentin and CD68 but lacked expression of follicular dendritic cell markers and actin. Epstein-Barr virus (EBV) genome was identified in the epithelioid and spindle cell population by in situ hybridization using probes specific for EBV-encoded RNAs (EBER1 and EBER2). Southern blot analysis of digested DNA extracted from the tumor using an EBV-specific probe (XhoI) demonstrated the presence of a single high-intensity band, indicative of EBV monoclonality. While there have been 2 previous reports of hepatic IPTs containing a monoclonal population of EBV-infected tumor cells, this is the first report of such an association occurring in the spleen. The presence of clonal EBV DNA suggests some splenic IPTs may be true neoplasms.

Key Words:
  • Inflammatory pseudotumor
  • Spleen
  • Clonal Epstein-Barr virus