Pediatric extranasal, EBV-negative, extranodal natural killer/T-cell lymphoma; case report

Extranodal natural killer/T-cell lymphomas (ENKTCL) are rare, aggressive neoplasms primarily occurring in adults of Asian or Native American descent. Most are associated with Epstein-Barr virus (EBV) and originate in the nasopharyngeal region. We present the case of a fulminant, disseminated, non-nasal, EBV negative, seemingly de novo ENKTCL in an infant who presented with a five-day history of progressive abdominal distention, respiratory distress, irritability, non-bilious emesis, right eye proptosis, and pallor. Initial labs revealed leukocytosis, anemia and thrombocytopenia. Computerized axial tomography revealed densities in the right optic nerve, superior orbits, thymus, posterior mediastinum, pericardium, myocardium, axillary lymph nodes, adrenal glands, kidneys and pancreas. The patient rapidly deteriorated, and despite extensive resuscitation, ultimately died. Autopsy revealed tumors in many organs, displaying vascular damage and tissue necrosis with lymphoid infiltration. The likely diagnosis of EBV-negative ENKTCL was confirmed after further histologic analysis. Pediatric ENKTCL is a particularly uncommon tumor, especially given our patient's very young age and EBV-negative status. Determining the best treatment regimen for these children is challenging due to limited data. Treatment typically consists of combination chemotherapy and radiation, but targeted therapies are being explored with promising results.