Extranodal NK/T-cell Lymphoma, Nasal Type

Abstract

Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is uncommon in the United States. We report 73 patients with ENKTL, including 49 men and 24 women (median age, 46 y). Sixty-three patients had nasal/upper aerodigestive tract disease; 10 had extranasal disease involving skin, small intestine, epiglottis, testis, adrenal glands, kidney, and breast. Complete staging data were available for 68 patients: 44 stage I/II and 24 stage IV. Fifteen of 69 (22%) had lymphadenopathy and 10/63 had bone marrow involvement. Histologically, 67/73 (92%) showed necrosis, and 48/70 (69%) had an angiocentric/angiodestructive growth pattern. The neoplastic cells showed a wide spectrum: medium sized (n=34), mixed small and large (n=21), large (n=13), and small (n=5). In situ hybridization for Epstein-Barr virus-encoded small RNA was positive in every case. Immunohistochemical studies showed expression of cytotoxic markers (100%), T-bet (96%), CD2 (96%), CD3 (93%), CD56 (90%), and ETS-1 (64%). Ki-67 was ≥60% in 46% cases. Therapy was known for 64 patients; 14 received only chemotherapy, 8 radiation alone, and 42 received combined radiation and chemotherapy. Median survival was 4.2 years, and 5-year overall survival was 46% (median follow-up, 3.8 y). Extranasal disease, high International Prognostic Index score, and high proliferation rate correlated with poorer prognosis. We conclude that ENKTL cases in the United States are similar to those reported in Asia and other countries. Absence of the angiocentric/angiodestructive pattern and presence of lymphadenopathy, features underemphasized in the literature, occurred in appreciable subsets of patients. The International Prognostic Index score, anatomic site of disease, and proliferation rate had prognostic value in this patient cohort.

A phase 2 study of methotrexate, etoposide, dexamethasone, and pegaspargase chemotherapy for newly diagnosed, relapsed, or refractory extranodal natural killer/T‐cell lymphoma, nasal type: a multicenter trial in Northwest China

“…Although patients whose tumors had ≥60% cells positive for Ki‐67 had a lower 3‐cycle rate of CR and lower 1‐year OS and PFS than did those whose tumors had <60% positive cells, the results were not statistically significant, and univariate analysis determined that a high Ki‐67 expression was not associated with a poor survival. This finding differs from those of previous studies 57, 58, 59, 60, 61, 62. Similarly, IPI score, disease stage, and expression of β2‐microglobulin and CD56, which have all been shown to be associated with survival for patients with nasal or extranasal NKTCL,59, 63, 64, 65, 66 were not associated with survival in our study.…”

“…This finding differs from those of previous studies 57, 58, 59, 60, 61, 62. Similarly, IPI score, disease stage, and expression of β2‐microglobulin and CD56, which have all been shown to be associated with survival for patients with nasal or extranasal NKTCL,59, 63, 64, 65, 66 were not associated with survival in our study. Additional research will be needed to determine which clinicopathological factors best predict the success of MESA chemotherapy.…”

Beneath the Retinal Pigment Epithelium: Histopathologic Findings in Metastatic Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

“…It commonly presents in adult men, especially in Asia and Central America, but there are infrequent reports of cases in Europe and the USA [17]. Seventy to ninety percent of ENKTCLs originate in the nasopharynx, although disseminated and extranasal disease also occurs [2, 3, 12, 18], as it did in our patient. This malignancy is characterized by an aggressive angiodestructive growth pattern and tumor necrosis [18] as well as a pathogenic link to EBV [19-22].…”

“…Seventy to ninety percent of ENKTCLs originate in the nasopharynx, although disseminated and extranasal disease also occurs [2, 3, 12, 18], as it did in our patient. This malignancy is characterized by an aggressive angiodestructive growth pattern and tumor necrosis [18] as well as a pathogenic link to EBV [19-22]. In our patient, the histologic appearance of the lymphomatous infiltrates and the expression of EBV by in situ hybridization helped us to confirm the diagnosis of ENKTCL, nasal type [18].…”

“…This malignancy is characterized by an aggressive angiodestructive growth pattern and tumor necrosis [18] as well as a pathogenic link to EBV [19-22]. In our patient, the histologic appearance of the lymphomatous infiltrates and the expression of EBV by in situ hybridization helped us to confirm the diagnosis of ENKTCL, nasal type [18]. In addition, the lymphoma cells uniformly expressed the T-cell marker CD3 and the cytotoxic granule-associated proteins granzyme B and TIA-1, and a majority of the cells were positive for CD7 and CD30.…”