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
Journal
Applied Immunohistochemistry and Molecular Morphology
Journal Volume
24
Journal Issue
2
Pages
105-111
Date Issued
2016
Author(s)
Abstract
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. Copyright ? 2015 Wolters Kluwer Health, Inc. All rights reserved.
SDGs
Other Subjects
CD2 antigen; CD20 antigen; CD3 antigen; CD56 antigen; CD7 antigen; cyclophosphamide; doxorubicin; granzyme B; prednisolone; T cell intracellular antigen 1; T lymphocyte antigen; T lymphocyte receptor gamma chain; unclassified drug; vincristine; adult; aged; cancer adjuvant therapy; cancer combination chemotherapy; cancer patient; cancer radiotherapy; clinical article; clonal variation; comparative study; Conference Paper; consultation; cutaneous T cell lymphoma; diagnostic error; disease association; disease course; disease duration; Epstein Barr virus; female; follow up; gene rearrangement; human; human tissue; immunohistochemistry; in situ hybridization; Japanese (people); large cell lymphoma; male; middle aged; multimodality cancer therapy; mycosis fungoides; NK T cell lymphoma; patient referral; peripheral T cell lymphoma; primary cutaneous extranodal natural killer t cell lymphoma; primary tumor; priority journal; retrospective study; skin nodule; subcutaneous nodule; subcutaneous t cell lymphoma; Taiwanese; world health organization; diagnostic error; differential diagnosis; Epstein Barr virus; Epstein-Barr Virus Infections; genetics; in situ hybridization; Lymphoma, Large-Cell, Anaplastic; Lymphoma, T-Cell, Cutaneous; Lymphoma, T-Cell, Peripheral; natural killer cell; patient referral; physiology; Skin Neoplasms; Adult; Aged; Diagnosis, Differential; Diagnostic Errors; Epstein-Barr Virus Infections; Female; Herpesvirus 4, Human; Humans; Immunohistochemistry; In Situ Hybridization; Killer Cells, Natural; Lymphoma, Large-Cell, Anaplastic; Lymphoma, T-Cell, Cutaneous; Lymphoma, T-Cell, Peripheral; Male; Middle Aged; Referral and Consultation; Retrospective Studies; Skin Neoplasms
Publisher
Lippincott Williams and Wilkins
Type
conference paper