Characteristics and Outcome of Extranodal NK/T-cell Lymphoma in North America: A Retrospective Multi-Institutional Experience

Extranodal natural killer/T-cell lymphoma is rare in the Western world. Clinical characteristics and outcomes remain unclear. We report on 121 cases from major centers in North America. The majority of patients present with early-stage disease, and CMT appears to provide optimal outcomes for appropriate candidates. Patients generally had poor outcomes, regardless of race/ethnicity, highlighting need for further advances in the field. Background: Extranodal natural killer/T-cell lymphoma (ENKTL) is rare and clinical data from non-Asian countries are lacking. It is unclear whether outcomes and disease natural history is similar to reported Asian series. We assessed characteristics and outcomes of patients with ENKTL from major North American centers. Patients and Methods: We retrospectively identified patients with newly-diagnosed CD56 + ENKTL and studied disease characteristics and clinical outcomes. Results: One hundred and twenty-one patients with ENKTL diagnosed between June 1990 and November 2012 were identified. Eighty-three patients (69%) had stage I/II disease and were treated with combined modality therapy (CMT) (n = 53), chemotherapy alone (CT) (n = 14) or radiotherapy alone (RT) (n = 16). Thirty-eight patients (31%) had stage III/IV disease and were treated with CMT (n = 12), CT (n = 23), or RT (n = 3). The median follow-up for the entire cohort was 51 months. Patients with stage I/II disease, compared to those with stage III/IV disease, had superior 2-year progression free survival (PFS) 43% vs 19% (P =.03) and overall survival (OS) 59% vs. 29% (P=.004). Outcomes were similar for stage I/II patients who received CMT vs. RT alone with 2-year PFS (53% vs. 47%; P=.91) and OS (67% vs. 67%; P=.58). No significant differences in outcomes were noted based on race/ethnicity. Conclusions: This series represents a large experience of ENKTL treated at several major North American academic centers. Our data are consistent with Asian studies: (1) majority of patients present with early-stage disease; (2) overall poor outcome regardless of race/ethnicity; (3) CMT likely yields favorable outcomes for suitable candidates with early-stage disease.

Automated summary

Extranodal natural killer/T-cell lymphoma is rare in the Western world, with most patients presenting with early-stage disease. Regardless of race/ethnicity, patients generally have poor outcomes, highlighting the need for further research and advancements in the field.