Cutaneous EBV-positive γδ T-cell lymphoma vs. extranodal NK/T-cell lymphoma: a case report and literature review

J Cutan Pathol. 2013 Mar;40(3):310-6. doi: 10.1111/cup.12066. Epub 2012 Dec 14.

Abstract

Primary cutaneous γδ T-cell lymphoma and extranodal natural killer (NK)/T-cell lymphoma (ENKTL), nasal type are two distinct lymphoma entities in the World Health Organization (WHO) classification. We report the case of an aggressive cutaneous lymphoma of γδ T-cell origin showing overlapping features of both lymphomas. A 78-year-old female presented with confluent erythematous plaques with ulcerations over her right thigh. Microscopically, section of the skin showed a diffuse dermal and subcutaneous lymphocytic infiltration with tumor necrosis and angioinvasion. The medium- to large-sized tumor cells expressed CD3, CD8, cytotoxic molecules and T-cell receptor (TCR)-γ but not CD4, CD20, CD30, CD56 or βF1. In situ hybridization for Epstein-Barr virus-encoded mRNA (EBER) was diffusely positive. Polymerase chain reaction-based clonality assay showed a clonal TCR-γ chain gene rearrangement. The features compatible with γδ T-cell lymphoma include dermal and subcutaneous involvements, cytotoxic phenotype, expression of TCR-γ, as well as an aggressive course. On the other hand, the diffuse EBER positivity, angioinvasion, tumor necrosis and cytotoxic phenotype may also fit in the diagnosis of an ENKTL of T-cell lineage. We review the literature on EBER-positive γδ T-cell lymphoma and discuss the diagnostic dilemma using the current WHO classification system.

Keywords: Taiwan; cutaneous lymphoma; extranodal natural killer/T‐cell lymphoma; peripheral T‐cell lymphoma; γδ T‐cell; γδ T‐cell lymphoma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Epstein-Barr Virus Infections* / classification
  • Epstein-Barr Virus Infections* / metabolism
  • Epstein-Barr Virus Infections* / pathology
  • Female
  • Gene Expression Regulation, Neoplastic
  • Gene Expression Regulation, Viral
  • Herpesvirus 4, Human*
  • Humans
  • Lymphoma, T-Cell, Cutaneous* / classification
  • Lymphoma, T-Cell, Cutaneous* / metabolism
  • Lymphoma, T-Cell, Cutaneous* / pathology
  • Lymphoma, T-Cell, Cutaneous* / virology
  • Neoplasm Proteins / metabolism
  • RNA, Viral / metabolism
  • Receptors, Antigen, T-Cell, gamma-delta*
  • Skin Neoplasms* / classification
  • Skin Neoplasms* / metabolism
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / virology
  • World Health Organization

Substances

  • Epstein-Barr virus encoded RNA 1
  • Neoplasm Proteins
  • RNA, Viral
  • Receptors, Antigen, T-Cell, gamma-delta