Concurrent presentation of distinct extranodal and splenic marginal zone lymphomas

Marginal zone lymphomas (MZLs) encompass extranodal, splenic, and nodal subtypes that are thought to clonally arise from antigen-exposed B-cells. Here, we present the case of a patient with a concurrent diagnosis of extranodal marginal zone lymphoma (EMZL) of the ocular adnexa and splenic marginal zone lymphoma (SMZL). These were identified on a core biopsy of the ocular adnexa and a bone marrow biopsy, respectively. While sequential occurrences of MZLs have been previously documented, this represents a concurrent diagnosis of two distinct MZLs.