Extranodal marginal zone lymphoma (EMZL) is a rare cancer of B-lymphocyte origin typically found in the gastrointestinal tract within the mucosa-associated lymphoid tissue. EMZL accounts for 5% of all non-Hodgkin's lymphomas and has been reported to affect the central nervous system in several select case reports. Here, we describe a rare case of EMZL presenting as an isolated skull mass, and review current management of EMZL. To our knowledge, this is the first reported patient of isolated EMZL of the skull.
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