Extranodal NK/T‐Cell Lymphoma, Nasal Type of Head and Neck in a Pediatric Population: A Systematic Review

Objective This review aims to identify and evaluate the management options for the extranodal NK/T‐cell lymphoma, nasal type of head and neck in the pediatric population. Methods The study was conducted based on a comprehensive literature review from 2009 to 2025. The following keywords were used: extranodal NK/T cell lymphoma and children and head and neck. Results The clinical data, including sex, age, symptoms, localization at presentation, correlation with EBV infection, treatment, and its outcome, were analyzed. A total of 64 patients were evaluated. Among these cases, the nasal site was the most involved region. Positive results for EBV infection were present in 100% of patients. The mortality rate in this study was 25%. Conclusions This systematic review highlights a clear male predominance, a pronounced geographic concentration of cases in East Asia, and underscores the diagnostic importance of EBV positivity in the diagnosis of ENKTL. In the case of the chronic course of destructive processes in aerodigestive organs with no improvement after standard treatment, NK‐cell lymphomas should be considered in the differential diagnosis. Multimodal treatment strategies, particularly asparaginase‐based chemotherapy with or without radiotherapy, are associated with improved outcomes, although disease‐related mortality remains substantial. Given the limited number of pediatric cases and the heterogeneity of available data, further prospective, multicenter studies with standardized diagnostic and therapeutic approaches are essential to optimize management and improve long‐term survival.