Treatment of localized extranodal NK/T cell lymphoma, nasal type: a systematic review

Abstract

Extranodal natural killer/T cell lymphoma (ENKTL), nasal type, presents predominantly as a localized disease involving the nasal cavity and adjacent sites, and the treatment of localized nasal ENKTL is a major issue. However, given its rarity, there is no standard therapy based on randomized controlled trials and therefore a lack of consensus on the treatment of localized nasal ENKTL. Currently recommended treatments are based mainly on the results of phase II studies and retrospective analyses. Because the previous outcomes of anthracycline-containing chemotherapy were poor, non-anthracycline-based chemotherapy regimens, including etoposide and l-asparaginase, have been used mainly for patients with localized nasal ENKTL. Radiotherapy also has been used as a main component of treatment because it can produce a rapid response. Accordingly, the combined approach of non-anthracycline-based chemotherapy with radiotherapy is currently recommended as a first-line treatment for localized nasal ENKTL. This review summarizes the different approaches for the use of non-anthracycline-based chemotherapy with radiotherapy including concurrent, sequential, and sandwich chemoradiotherapy, which have been proposed as a first-line treatment for newly diagnosed patients with localized nasal ENKTL.

Metabolic activity of extranodal NK/T cell lymphoma on 18F-FDG PET/CT according to immune subtyping

“…Extranodal NK/T cell lymphoma (ENKTL), nasal type, is a rare type of lymphoma that ordinarily involves midline areas of the nasal cavity, oral cavity, and adjacent sites 1 . Some patients with ENKTL demonstrate distant nodal or extranodal involvement, which is associated with significantly worse survival outcomes 2 , 3 .…”

Plasma circulating tumor DNA assessment reveals KMT2D as a potential poor prognostic factor in extranodal NK/T-cell lymphoma

“…Extranodal NK/T-cell lymphoma, nasal type (ENTKL), is an aggressive extranodal lymphoma of NK-cell or T-cell lineage, which is highly aggressive and heterogeneous disease, with predominance in males [1][2][3][4]. Current treatment strategies (such as the combination of chemotherapy, radiotherapy, targeted therapy) can improve the complete remission of patients, but most will ultimately relapse and progress [5][6][7].…”

Educational Case: Extranodal NK/T-Cell Lymphoma, Nasal Type

“…The combined approach of non-anthracycline-based chemotherapy with radiotherapy is currently recommended as a first-line treatment for ENKTL-NT. 5,11 One meta-analysis summarized that in 7 retrospective cohort studies with 1593 patients included, compared with induction chemotherapy followed by radiotherapy, upfront radiotherapy significantly improved overall survival of patients with limited stage ENTKL. 12,13…”

“…The combined approach of non-anthracycline-based chemotherapy with radiotherapy is currently recommended as a first-line treatment for ENKTL-NT. 5,11 One meta-analysis summarized that in 7 retrospective cohort studies with 1593 patients included, compared with induction chemotherapy followed by radiotherapy, upfront radiotherapy significantly improved overall survival of patients with limited stage ENTKL. 12,13 With further understanding of the specific protein expression within ENKTL-NT, new drugs targeting CD38 (daratumumab, naked anti-CD38 antibody), CD30 (brentuximabvedotin, anti-CD30 antibody conjugated with auristatin E), programmed death-ligand 1 (PD-L1), and programmed death 1 (PD-1) show promising future.…”