Extranodal natural killer/T cell lymphoma, nasal type: an update on diagnosis and treatment / 中华实用儿科临床杂志

ABSTRACT

Extranodal natural killer(NK)/T cell lymphoma, nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma, closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis, and is prevalent in East Asia and South America.Clinicopathologically, ENKTL is characterized by diffuse tumor cell infiltration, angiocentral destructive growth pattern, and prominent tissue necrosis.Typically, ENKTL presents with extranodal involvements, particularly the upper aerodigestive tract, such as nasal cavity, nasopharynx, paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis, refined tumor staging and risk assignment, and treatment strategies including local radiotherapy, concurrent chemoradiotherapy and sandwich therapy.Accordingly, response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors, though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.