Epstein-Barr virus-related atypical lymphoproliferative disorders in Waldeyer's ring: a clinicopathological study of 9 cases

Pathobiology. 2010;77(4):218-24. doi: 10.1159/000301154. Epub 2010 Jul 7.

Abstract

Background and study aim: Because of the small biopsy specimens in the Waldeyer's ring (WR) the differential diagnosis between Epstein-Barr virus (EBV)-associated lymphoproliferative disorder (LPD) and malignant lymphoma is occasionally difficult. We report here clinicopathological, immunohistochemical and genotypic findings of 9 cases of EBV-associated LPDs in WR.

Patients and methods: Using formalin-fixed paraffin-embedded sections, histological analyses, immunohistochemistry, in situ hybridization and polymerase chain reaction were performed.

Results: Clinically, all 9 cases showed more than one atypical clinical finding of infectious mononucleosis including absence of systemic symptoms, absence of atypical lymphocytosis and age over 30 years. Histologically, 3 types were delineated: (1) Hodgkin lymphoma-like (n = 1), (2) T cell/histiocyte-rich large B cell lymphoma-like (n = 4), and (3) marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT)-like (n = 4). The in situ hybridization study demonstrated EBV-encoded small RNA (EBER)+ cells in all 9 lesions. The immunohistochemical and flow cytometry study demonstrated the reactive nature of the B cells in all 9 lesions. However, 3 of our 7 cases examined demonstrated immunoglobulin heavy chain gene rearrangement on PCR study. There was no development of B cell lymphoma in any of the 3 lesions demonstrating IgH rearrangement.

Conclusion: EBV-associated LPDs of the WR showed marked histological diversity. Among these, a MALT-like pattern was frequently seen. Marginal zone B cell lymphoma frequently affects WR. We emphasized that EBV-associated LPD should be added to the differential diagnosis of primary tonsillar MALT-type lymphoma.

MeSH terms

  • Adult
  • B-Lymphocytes / pathology
  • Female
  • Herpesvirus 4, Human* / genetics
  • Histiocytes / pathology
  • Hodgkin Disease / complications
  • Hodgkin Disease / pathology
  • Humans
  • Hyperplasia / complications
  • Hyperplasia / pathology
  • Immunohistochemistry
  • In Situ Hybridization
  • Lymphoma / complications
  • Lymphoma / pathology
  • Lymphoma, B-Cell / complications
  • Lymphoma, B-Cell / pathology
  • Lymphoma, B-Cell, Marginal Zone / complications
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoproliferative Disorders / complications
  • Lymphoproliferative Disorders / pathology*
  • Lymphoproliferative Disorders / virology*
  • Male
  • Middle Aged
  • Precancerous Conditions / complications
  • Precancerous Conditions / pathology