Extranodal hematopoietic neoplasms and mimics in the head and neck: an update

The head and neck region is a common site for extranodal lymphomas, second only to the gastrointestinal tract; and 12% to 15% of all head and neck tumors are lymphomas. Non-Hodgkin lymphomas are most common, and Hodgkin lymphoma occurs rarely at extranodal sites in the head and neck. Most non-Hodgkin lymphomas of the head and neck region are of B-cell lineage, and the Waldeyer ring is the most common site. Head and neck lymphomas have distinctive epidemiological and clinicopathologic features, including an association with immunosuppression, infectious organisms, or autoimmune disorders; site-specific differences (eg, thyroid gland versus ocular adnexa) for common lymphomas, such as extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue; and genetic differences that provide insights into etiology. Furthermore, the diagnosis of non-Hodgkin lymphomas at extranodal sites implies differences in prognosis and therapeutic implications with lymphomas at nodal sites. In this review, we discuss various types of non-Hodgkin lymphomas and Hodgkin lymphoma, focusing on unique aspects related to the head and neck region. We also discuss a number of newer entities that are clinically indolent as well as mimics of lymphoma that can occur in the head and neck region, including infectious mononucleosis, Kikuchi-Fujimoto disease, Kimura disease, Castleman disease, and immunoglobulin G4-related disease.