Two-year-old female with EBV-positive diffuse large B-cell lymphoma and subsequent CNS involvement with neurolymphomatosis.

We describe a case of Epstein-Barr virus (EBV) positive, diffuse large B-cell lymphoma in a 2-year-old female who went on to develop relapsed/refractory central nervous system (CNS) disease, manifesting as cranial nerve neurolymphomatosis. Although her atypical presentation was thought to be associated with an immune deficiency, extensive work-up was negative. Despite subsequent treatment with third-party EBV-specific cytotoxic T-lymphocytes, she died of progressive disease. This case report raises questions as to whether tailored treatment approaches should be considered for atypical presentations of pediatric lymphoma (e.g., CNS and virus-associated).