CD3+/CD56+ EBV+ neoplasms in the nose and upper aerodigestive tract: potential misdiagnosis of plasma cell malignancies
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LETTER TO THE EDITOR
CD3+/CD56+ EBV+ neoplasms in the nose and upper aerodigestive tract: potential misdiagnosis of plasma cell malignancies as NK/T cell lymphoma Rex Au-Yeung 1 & Florence Loong 1 & Yok-Lam Kwong 2 Received: 5 August 2020 / Accepted: 24 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Dear Editor, Lymphomas are, second to epithelial cancers, the most common malignancies in the head and neck region. They are mainly of B cell lineage [1]. Occasional cases are of T cell or NK-cell lineage, and can be nodal or extranodal. For extranodal T cell or NK-cell neoplasms, the important ones are NK/T cell lymphoma and CD30-positive T cell lymphoproliferative diseases [1]. NK/T cell lymphomas are mainly localized to the nose and upper aerodigestive tract [2]. Lymphoma cells are universally infected by the Epstein-Barr virus (EBV). They are positive for CD3, CD56, and cytotoxic molecules; and EBV early RNA (EBER) on in-situ hybridization. Patient 1 was a 37-year-old man referred for management of an oral NK/T cell lymphoma. He had a two-month history of a right mandibular mass, which extended into the oral cavity. A positron emission tomography computed tomography (PET/CT) scan showed a mass destroying the mandible, associated with multiple skeletal metastases. The initial biopsy was interpreted to show an EBV-positive peripheral T cell lymphoma (PTCL) or NK/T cell lymphoma. Bone marrow examination showed EBER-positive atypical lymphoid cells, consistent with lymphomatous involvement. On referral, the right mandibular biopsy was reviewed (Fig. 1). Neoplastic cells showed large nuclei, dispersed chromatin, and small nucleoli. On paraffin sections, they were
* Yok-Lam Kwong [email protected] Florence Loong [email protected] 1
Department of Pathology, University of Hong Kong, Queen Mary Hospital, Hong Kong, Hong Kong
2
Department of Medicine, University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong, China
positive for CD3 and EBER, but negative for CD56, TIA-1, CD30, and CD20. On cryostat sections, they were negative for surface-CD3, CD19, CD22, and T cell receptors (TCR, alpha/ beta, and gamma/delta). Features were unusual for PTCL (positivity only for cytoplasmic CD3-epsilon on paraffin section) and NK/T cell lymphoma (negativity for CD56 and TIA1). Polymerase chain reaction (PCR) for TCR genes did not show any clonal rearrangement. Additional immunostaining showed that neoplastic cells were diffusely positive for CD138 and epithelial membrane antigen, expressed kappa but not lambda light chain, and were negative for ALK and HHV-8. PCR showed clonal rearrangement of the immunoglobulin heavy chain gene. He was found to be positive for human immunodeficiency virus (HIV) 1. The diagnosis was revised to EBV+ plasmablastic lymphoma. He was treated with CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) and prophylactic intrathecal methotrexate. Remission was achieved, but the lymphoma relapsed about a year afterwards. Salvage chemotherapy was ineffectiv
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