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

• Published in: Journal of cutaneous pathology 2013-03, Vol.40 (3), p.310-316
• Main Authors: Yu, Wei‐Wen, Hsieh, Pin‐Pen, Chuang, Shih‐Sung
• Format: Article
• Language: English
• Subjects: Aged; cutaneous lymphoma; Epstein-Barr Virus Infections - classification; Epstein-Barr Virus Infections - metabolism; Epstein-Barr Virus Infections - pathology; extranodal natural killer/T‐cell lymphoma; 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; peripheral T‐cell lymphoma; Receptors, Antigen, T-Cell, gamma-delta; RNA, Viral - metabolism; Skin Neoplasms - classification; Skin Neoplasms - metabolism; Skin Neoplasms - pathology; Skin Neoplasms - virology; Taiwan; World Health Organization; γδ T‐cell; γδ T‐cell lymphoma
• ISSN: 0303-6987; 1600-0560
• DOI: 10.1111/cup.12066

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.