Primary cutaneous extranodal natural killer/T-cell lymphoma misdiagnosed as peripheral T-cell lymphoma: The importance of consultation/referral and inclusion of EBV in situ hybridization for diagnosis

Khin Than Win, Jau Yu Liau, Bo Jung Chen, Katsuyoshi Takata, Chiao Yun Chen, Chi Cheng Li, Cheng Hsiang Hsiao, Shih Sung Chuang

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7 Citations (Scopus)


Background: Primary cutaneous T-cell lymphomas (CTCL) are heterogenous extranodal non-Hodgkin lymphomas including a few distinct and provisional entities. Compared with the West, Asian populations have a relatively higher frequency of nonmycosis fungoides CTCL. Primary cutaneous extranodal natural killer/T-cell lymphoma (PC-ENKTL) is distinct from other CTCL by the presence of EBV association. Method: In our recent retrospective Asian study of PC-ENKTL, we identified 5 cases initially misdiagnosed as various CTCL. We fully characterized these cases with immunohistochemistry, EBV in situ hybridization, and clonality study for T-cell receptor (TCR) γ-chain gene (TRG). Results: The 5 patients included 3 males and 2 females with a median age of 45. All tumors were positive for EBER. Two cases were clonal for TRG gene rearrangement but without expression of βF1 or TCR-γ (TCR-silent T-cell origin), 1 tumor expressed TCR-γ (γδ T-cell origin), and the remaining 2 were polyclonal for TRG and negative for TCR expression (NK-cell origin). On the basis of the initial diagnoses (2 as peripheral T-cell lymphoma, unspecified, 2 as primary cutaneous anaplastic large-cell lymphoma, and 1 as subcutaneous panniculitis-like T-cell lymphoma), all patients received CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) chemotherapy with additional radiotherapy in 3. All patients experienced persistent disease or relapse despite treatment in a mean duration of 8.8 months (range, 1 to 12 mo). Conclusions: PC-ENKTL is rare and aggressive. These cases strongly demonstrate the importance of consultation/referral to experienced hematopathologists and the inclusion of EBER in the initial diagnostic work-up for patients with nonmycosis fungoides CTCL to avoid erroneous diagnosis and subsequent inadequate treatment of the patients.

Original languageEnglish
Pages (from-to)105-111
Number of pages7
JournalApplied Immunohistochemistry and Molecular Morphology
Issue number2
Publication statusPublished - 2016



  • Consultation
  • Cutaneous T-cell lymphoma
  • Epstein-Barr virus
  • Extranodal natural killer/T-cell lymphoma
  • Natural killer/T-cell lymphoma
  • Peripheral T-cell lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology
  • Histology

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