Primary Cutaneous Marginal Zone Lymphoma: A Comprehensive Review

Primary cutaneous marginal zone lymphoma (PCMZL) represents a subset of primary cutaneous B-cell lymphomas (PCBCL) and an even smaller subset of all cutaneous lymphomas. Due to PCMZL’s indolent nature, the differentiation between PCMZL and other more aggressive PCBCLs can be challenging and result in the delay of diagnosis and treatment. The diagnosis of PCMZL requires clinical evaluation combined with immunohistochemical analysis to differentiate it from other lymphoma and non-lymphoma pathologies. The condition’s wide range of treatment options, including novel therapies, have led to various outcomes for patients diagnosed with PCMZL. This comprehensive review article emphasizes the clinical presentation and the methods of diagnosis, including appropriate pathologic evaluation, differentiating factors from other non-PCMZL conditions, and current treatment options for PCMZL.