Extranodal natural killer/T-cell (ENK/T) lymphoma is a rare neoplasm, subcategorized into ENK/T-nasal (ENK/T-N) and ENK/T-nasal type (ENK/T-NT) lymphomas. ENK/T-NT lymphoma with initial presentation in the skin is known as primary cutaneous ENK/T-NT (PC-ENK/T-NT) lymphoma. The aim of this study was to investigate pathogenesis, genomic alterations, and prognosis of cutaneous ENK/T lymphomas to provide further insights into clinicopathologic features and genetic mechanism of lymphomagenesis. A retrospective case study of 5 white patients affected by ENK/T lymphoma (4 PC-ENK/T-NT and 1 ENK/T-N with cutaneous involvement) was performed. Most of the cases presented with multiple nodules and ulcerations localized on the extremities. A considerable percentage had disease in advanced stage with a 12-month survival rate of 40%. Genomic alterations were detected by array-based comparative genomic hybridization that showed gains of 1q, 7q and loss of 17p in the cases of PC-ENK/T-NT lymphomas and gain of 7q and loss of 9p, 12p, 12q in the case of ENK/T-N lymphoma. In conclusion, ENK/T lymphoma is a very aggressive entity, and, in our cases, the exclusively cutaneous presentation was not associated with a better prognosis. The results of our array comparative genomic hybridization analysis could be useful to better define the different ENK/T lymphoma subgroups with cutaneous involvement.
"A few studies have described the cytogenetic alterations based on limited numbers of fresh or frozen tissues, but no specific changes have been achieved [3–7]. Recently, gene expression profiling studies and array comparative genomic hybridization (CGH) analyses revealed that NK/TCL showed frequent 2q gain and 6q16-25 loss [8–11]. "
[Show abstract][Hide abstract] ABSTRACT: Extranodal NK/T-cell lymphoma, nasal type, is an aggressive mature NK-cell/T-cell lymphoma. Using array-based comparative genomic hybridization (array CGH) assays, we screened genomic alterations and potential candidate genes implicated in pathogenesis, progression, and prognosis. Our array CGH analysis detected an average of 83 chromosomal aberrations in 13 cases, ranging from 0 to 387. There were 177 recurrent chromosomal gains and 35 recurrent losses. Eleven gains and 14 losses were detected in more than 30 % of the cases, including gains of 3q26.1, 7q34, and 8q24.3 and losses of 15q24.2, 19q13.32, 5p13.2, and 14q21.1. The most common losses were observed in the 15q24.2 and 19q13.32 regions (9 cases, 69.2 %, respectively). Loss of 8p11.23 was associated with significant poor survival (P = 0.024). Five out of six patients with the loss of 8p11.23 died within 8 months after initial diagnosis with a median survival of 6 months. Several candidate genes were identified in the regions with frequent chromosomal aberrations, including ADAM3A (8p11.23) and GSTT1 (22q11.23). In summary, our studies detected recurrent genetic alterations in NK/T-cell lymphoma, some of which are associated with adverse prognosis. Some candidate genes in these regions may be involved in the pathogenesis and disease progression.
Medical Oncology 07/2014; 31(7):71. DOI:10.1007/s12032-014-0071-z · 2.63 Impact Factor
"On the other hand, some authors have suggested that nasal and extranasal NKTCLs behave alike at the same stage of the disease , . One publication addressing this subject has suggested that there are different genetic alterations in the two subgroups . Nasal NKTCL patients with PRF1 SNVs seem to behave more aggressively than those without it. "
[Show abstract][Hide abstract] ABSTRACT: NK/T-cell lymphoma (NKTCL) is the most frequent EBV-related NK/T-cell disease. Its clinical manifestations overlap with those of familial haemophagocytic lymphohistiocytosis (FHLH). Since PERFORIN (PRF1) mutations are present in FHLH, we analysed its role in a series of 12 nasal and 12 extranasal-NKTCLs. 12.5% of the tumours and 25% of the nasal-origin cases had the well-known g.272C>T(p.Ala91Val) pathogenic SNP, which confers a poor prognosis. Two of these cases had a double-CD4/CD8-positive immunophenotype, although no correlation was found with perforin protein expression. p53 was overexpressed in 20% of the tumoral samples, 80% of which were of extranasal origin, while none showed PRF1 SNVs. These results suggest that nasal and extranasal NKTCLs have different biological backgrounds, although this requires validation.
PLoS ONE 03/2014; 9(3):e91521. DOI:10.1371/journal.pone.0091521 · 3.23 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Breast lymphoma is uncommon, accounting for approximately 0.15% of malignant breast lesions. It usually originates from lymphocytes in breast parenchyma and ipsilateral axillary lymph nodes. In breast, diffuse large B-cell lymphoma is more common than T-cell lymphoma. Here, we report a rare case of cutaneous natural killer/T-cell (NK/T) lymphoma mimicking panniculitis of the breast, that was presented as a growing palpable mass and pain in both breasts of a 33-year-old male patient. Ultrasonographic appearance was extensive hyperechogenicity in subcutaneous fat layer of mammary areas, mimicking panniculitis or fat necrosis. Pathologically, a histologic subtype was extranodal natural killer/T-cell lymphoma, nasal-type.
European Journal of Radiology Extra 05/2011; 78(2). DOI:10.1016/j.ejrex.2011.03.003
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