THINK SINUSITIS? THINK PNEUMONIA? THINK AGAIN! A CASE OF EXTRANODAL NK/T-CELL LYMPHOMA, NASAL TYPE.

Abstract

Introduction Extranodal natural killer/T-cell lymphoma (ENKL), nasal type, is a rare, aggressive, peripheral lymphoma with uncertain cellular origins and infection of tumor cells with Epstein-Barr Virus (EBV). ENKL comprises 2-10% of primary non-Hodgkin's disease, and often presents with rhinosinusitis or a sinus mass; less often there is involvement of other extranodal sites, such as the lungs, gastrointestinal tract, or bone marrow. Case Description A 37 year-old man presented with a two week history of cough, dyspnea, congestion, facial pressure, fever, chills, night sweats, and weight loss. Chest CT showed areas with ground glass appearance and nodular opacities predominately in the peribronchial region without any lymphadenopathy. Sinus CT showed chronic inflammatory changes in the right maxillary sinus. The patient was diagnosed with pneumonia and started on antibiotics. Biopsies taken during bronchoscopy revealed atypical lymphoid cells with an NK-cell phenotype positive for CD3, CD7, CD56, TIA-1, granzyme, and perforin. His disease was complicated by hemophagocytic lymphohistiocytosis, which can occur in 3% of ENKL cases. Despite chemotherapy, the patient died. Autopsy confirmed an EBV+ extranodal NK/T-cell lymphoma, nasal type, involving the lungs, maxillary sinus, heart, kidneys, and liver with diffuse positivity for CD3, CD56, perforin, and variable positivity for EBV-latent membrane protein. Discussion ENKL is a rare, aggressive malignancy that is often diagnosed late due to an insidious presentation; our patient was presumed to have pneumonia. There is a dearth of ENKL cases that present with primarily pulmonary symptoms. Diagnosis is always confirmed with biopsy, which can be anatomically challenging to acquire. CT Scan of Patient's Maxillary Sinuses