Composite lymphoma: angiocentric T-cell lymphoma (CD8+ cytotoxic/supressor T-cell) and diffuse large B-cell lymphoma associated with EBV, and presenting clinically as a midfacial necrotizing lesion

Abstract

A composite lymphoma is defined as the simultaneous occurrence of two histologically different types of lymphomas situated in one anatomical location. Reports of composite B- and T-cell lymphomas, especially in the head and neck region, are rare. We describe a 76-year-old Taiwanese aboriginal female patient clinically presenting with a midfacial necrotizing lesion (MNL). Microscopic examination of the incisional biopsy specimen revealed extensive surface necrosis with infiltrates of inflammatory cells. Beneath the necrotic surface, there appeared to be two distinct populations of pleomorphic lymphoid cells exhibiting the characteristic features of the angiocentric distribution of the tumor cells and evidence of angiodestruction. Immunohistochemical staining revealed that these atypical lymphoid cells were positive for LCA, CD45, CD5, CD20, CD3ε, CD8, bcl-2 and bcl-6 and negative for CD56, CD4, CD68, keratin, S-100, kappa and lambda. Furthermore, these atypical lymphoid cells also expressed EBV-encoded nuclear RNAs (EBERs) following in situ hybridization. Therefore, this was a case of composite lymphoma: angiocentric T-cell lymphoma (ATCL) (CD8+ cytotoxic/suppressor T-cell) and diffuse large B-cell lymphoma (DLBL) associated with the Epstein–Barr virus (EBV) and presenting clinically as MNL.