# Extranodal Natural Killer (NK)/T-Cell Lymphoma Causing Severe Facial Destruction Refractory to Chemotherapy and Immunotherapy

## TL;DR

A rare and aggressive lymphoma caused severe facial destruction and was unresponsive to standard treatments, highlighting the need for early diagnosis.

## Contribution

This case report highlights the diagnostic challenges and aggressive nature of ENKTCL, emphasizing the importance of early histopathologic and EBV testing.

## Key findings

- The patient presented with rapidly progressive necrotic facial lesions refractory to chemotherapy and immunotherapy.
- Diagnosis was confirmed with histopathology and EBV-encoded RNA in situ hybridization.
- The case illustrates the severe clinical course and poor response to standard treatment regimens in advanced ENKTCL.

## Abstract

Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL), is a rare, highly aggressive Epstein-Barr virus (EBV)-associated non-Hodgkin lymphoma. Diagnosis is frequently delayed because early presentation is often non-specific and may mimic many other conditions, including disseminated fungal or atypical bacterial infections, autoimmune conditions such as vasculitis, and other cutaneous malignancies, such as peripheral T-cell lymphomas or melanoma. We present a case of extensive, rapidly progressive and highly destructive necrotic facial and cutaneous lesions, which remained undifferentiated despite initial diagnostic workup, necessitating travel to a quaternary medical center on a medical visa for further evaluation. A thorough workup, including a histopathologic evaluation along with a positive EBV-encoded RNA in situ hybridization, confirmed ENKTCL. The patient’s clinical course was marked by extensive local tissue destruction, EBV viremia, severe malnutrition, recurrent episodes of shock, and disease progression despite treatment with pegaspargase, gemcitabine, and oxaliplatin (P-GEMOX) and modified dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) chemotherapy and pembrolizumab. This case underscores the diagnostic challenges of ENKTCL and highlights the aggressive clinical course associated with delayed diagnosis and advanced-stage disease, emphasizing the importance of early histopathologic evaluation with EBV testing in patients with progressive necrotic midline lesions.