CD56-negative extranodal nasal type NK/T-cell lymphoma

Abstract

Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare lymphoma that occurs predominantly in Asian adults. In this report, we describe the clinical and pathologic features of an unusual aggressive lymphoid neoplasm in a child and review the literature on NK/T-cell lymphoma in children. The patient was a 4-year-old Native American male with facial swelling, lymphadenopathy, and fevers. Biopsy demonstrated neoplastic lymphoid cells that expressed CD3, CD8, TIA-1, and EBV-encoded RNA without CD56. The patient failed multiagent chemotherapy and died of therapy-related complications. This case represents an extranodal NK/T-cell lymphoma, nasal type, with an unusual lack of CD56.