Extra-nodal NK/T-cell lymphoma, nasal type (EN-NK/TCL-NT), is rare in the Western world. We launched the current single-institutional retrospective study with Institutional Review Board approval to better understand the disease. 43 EN-NK/TCL-NT patients treated from 1996 to 2014 were analyzed, including 10 (23%) Asians and 33 (76%) non-Asians. 19/26 (73%) early-stage patients received short-course chemotherapy followed by radiotherapy. 14/17 (82%) advanced-stage patients received primary chemotherapy. Complete response rate was significantly higher in the modified-SMILE group than the accelerated-CHOP group (80% vs. 30%, p = 0.015). The 2-year overall survival (OS) and progression-free survival (PFS) were 60% and 40%, respectively. Early-stage disease had significantly higher 2-year OS (87% vs. 21%) and PFS (56% vs.18%) than advanced-stage (p < 0.001). Ethnicity had no prognostic difference. EN-NK/TCL-NT in non-Asians shared similar disease characteristics and treatment outcomes with Asians. Most early-stage patients have achieved durable remissions. Management of advanced-stage disease remains challenging, with frequent progression and high mortality.
Keywords: Drug therapy; SMILE; ethnology; extranodal NK-T-cell lymphomas; radiotherapy.