Nasal T-cell lymphoma: a clinicopathologic entity associated with peculiar phenotype and with Epstein-Barr virus

Abstract

Recent evidence has shown that most nasal lymphomas (NL) are associated with a T-cell phenotype and are thus called nasal T-cell lymphomas (NTCL), but little information is available about the T-cell receptor (TCR) expression. The presence of Epstein-Barr virus (EBV) genome has been recently reported in NTCL in Oriental populations in which NL and EBV-associated tumors are more common and in occasional Occidental cases. This prompted us to investigate lymphoma biopsies from 7 non- Oriental patients with NTCL for the expression of natural killer (NK) and T-cell antigens, including TCR proteins, for the presence of EBV- encoded latent membrane protein (LMP) using immunohistochemistry and for the presence of EBV DNA and Epstein-Barr early region (EBER) RNA using in situ hybridization (ISH). Six cases displayed a CD3-, TCR alpha beta-, TCR gamma delta-, CD2+, CD7+, CD5-, CD4-, CD8-, CD56+ phenotype, suggesting that these tumors may be peripheral T-cell lymphomas (PTCL) with extensive loss of T-cell antigens and expression of the NK-cell (CD56) antigen or, alternatively, NK-cell neoplasias. The remaining case was a gamma delta PTCL, as shown by the CD3+, TCR gamma delta+ phenotype and the biallelic gamma and delta TCR gene rearrangements. Using ISH, EBER RNA transcripts were detected in tumor cells in all cases and EBV DNA was shown in the 6 tested cases. In all cases, tumor cells expressed LMP. These findings support the concept that NTCL constitute a distinct group of lymphomas that, in addition to their peculiar clinical features, exhibit an unusual TCR “silent” CD56+ or TCR gamma delta+ phenotype and harbor the EBV. In view of the LMP transforming potential, these data suggest that EBV may play a role in the pathogenesis of NTCL.

Cutaneous presentation of nasal/nasal type T/NK cell lymphoma: clinicopathological findings of four cases

“…Interestingly, Jaffe et al 17 described rare cases that may present in the skin or soft tissue and relapse in the nose. In our review of the literature between 1983 and 1996, we found only 17 clear cases, 3,7,8,11,18,[21][22][23][24][25][26][27][28] of peripheral T-cell lymphoma with skin as well as nose metastasis. In six cases, 7,18,23,27,28 the lymphoma clearly proceeded from the skin.…”

Cutaneous dissemination of nasal NK/T-cell lymphoma with bone marrow, liver and lung involvement

“…1-10 NKTCL shows high prevalence in people of Asian decent and commonly presents with midline facial destructive disease. [1][2][3][4][5] Morphologically, NKTCL is characterized by a broad cytological spectrum; the atypical cells may be small or medium-sized cells, large atypical and hyperchromatic cells or a mixture of these types with coagulation necrosis of dermis and subcutis. Virtually all cases of NKTCL are positive for EBV.…”

“…Because the nasal area is the most common site of presentation and CD56+ NK cells expressing T-cell immunophenotype are the most likely lineage, nasal NKTCL has been thought to be an appropriate term. [1][2][3][4][5] Tumours with identical morphology, immunophenotype, and genotype presenting in extra-nasal areas were given the name of nasal type NKTCL. 9,10 Nasal NKTCL is mostly localized to the nasal areas but shows a propensity to spread or recur in the certain predilection sites such as skin, palate, tonsil, testis, gastrointestinal tract and other soft tissues.…”

“…9,10 Nasal NKTCL is mostly localized to the nasal areas but shows a propensity to spread or recur in the certain predilection sites such as skin, palate, tonsil, testis, gastrointestinal tract and other soft tissues. [3][4][5][6] Primary cutaneous nasal type NKTCL shares features with subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Both can present as cutaneous nodules or ulcers; epidermotropism is uncommon to each; both are associated with haemophagocytic syndrome.…”

Maintenance and Characterization of an Epstein Barr Virus‐infected CD56‐negative T Cell Lymphoma

“…Epstein-Barr virus (EBV) is associated with various lymphoid malignancies, including Burkitt's lymphoma, 1) lymphoma occurring in immunocompromised patients, 2) Hodgkin's disease, 3) pyothorax-associated lymphoma, 4,5) nasal and nasal type T/Natural Killer (T/NK) cell lymphoma (NT/NKL), 6) NK cell leukemia/lymphomas, [7][8][9] Tcell lymphoma, 10) and a few other lymphoma subtypes. B cell lymphoma predominates in EBV-associated lymphomas, while some portions are reported to be of T and/or NK cell lineage.…”