Introduction

Extranodal natural killer/T-cell lymphoma, nasal type, is a rare and aggressive malignancy of the midline facial structures, strongly associated with Epstein–Barr virus-driven oncogenesis. Because it may mimic infectious, inflammatory, granulomatous, or necrotizing disorders, diagnosis is often delayed until progressive angiocentric tissue destruction becomes clinically evident.

Case report

We report the case of a 40-year-old woman whose initially subtle nasal obstruction and discharge progressed to severe midfacial destruction, with a friable mass eroding the septum and palate. Histopathological examination showed pandermal, subcutaneous, and angioinvasive atypical lymphoid infiltrates. Immunohistochemistry and Epstein–Barr virus-encoded RNA <i><i>in situ</i></i> hybridization confirmed Epstein–Barr virus-associated cytotoxic T/natural killer-cell lineage, establishing the diagnosis. The patient was started on an L-asparaginase-based regimen, in line with current therapeutic approaches that favor non-anthracycline protocols.

Conclusions

This case highlights diagnostic challenges of extranodal natural killer/T-cell lymphoma, nasal type, its ability to mimic granulomatous and necrotizing conditions, and the essential role of early biopsy with comprehensive immunophenotyping. The case is presented together with a review of the biology, diagnostic pathway, and evolving therapeutic landscape of this aggressive neoplasm.