Resumen
Extranodal NK/T cell lymphoma (ENKTCL) is an aggressive lymphoma associated with Epstein-Barr virus (EBV) infection that occurs mainly in Asian and Latin American populations. In the last decade, the genetic landscape of ENKTCL has been characterized comprehensively using next-generation sequencing (NGS). This and similar high-throughput approaches revealed that these lymphomas are distinguished by frequent gene mutations leading to activation of the JAK-STAT pathway, and mutations in other genes such as BCOR, DDX3X and TP53. This review aims to provide a comprehensive overview about the role of EBV infection and a comparison of the EBV strains and LMP1 variants among different populations. Moreover, a brief summary of the ENKTCL genetic landscape is presented, highlighting the main therapeutically targetable pathways in ENKTCL oncogenesis: the JAK-STAT signaling pathway, the immune response evasion, MYC overexpression, as well as epigenetic alterations.