Non-Hodgkin's lymphoma of the oral cavity and maxillofacial region: a pathologist viewpoint.

INTRODUCTION: Non-Hodgkin lymphomas of the oral and maxillofacial regions are uncommon malignancies. They usually have progressive course and dismal outcome. Areas covered: This review provides an update about the clinicopathologic, immunophenotypic, and molecular features of these lymphomas. Some relevant case reports are presented. Expert commentary: The lymphomas of the oral and maxillofacial regions involve the tongue, gingiva, hard palate, maxilla, mandible, Waldeyer's ring, palatine, and lingual tonsils. They include diffuse large B-cell lymphoma (DLBCL), T-cell lymphoma, Burkitt lymphoma (BL), natural killer/T-cell lymphoma (NKTCL), marginal zone lymphoma, plasmablastic lymphoma (PBL), mantle cell lymphoma, and lymphoblastic lymphoma. They are more common in males than in females. They commonly present as mass lesions covered by an intact or ulcerated mucosa. Some lymphomas are associated with viral infections (Epstein-Barr virus or HIV) and specific genetic alterations. Each subtype has its specific clinicopathologic features and the most common subtypes include DLBCL, BL, NKTCL, and PBL. DLBCL is the most common oral lymphoma. BL is a rapidly proliferating mature B cell neoplasm almost always presenting in extranodal sites. Nasal-type NKTCL presents as a locally destructive, midfacial necrotizing lesion. PBL is a rare, highly aggressive disease that is commonly associated with immunodeficiency conditions.