Introduction: Extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue is an indolent lymphoma originating from marginal zone B-cells and associated with chronic inflammation. EMZL demonstrates distinct genomic alterations according to the primary extranodal site of disease but commonly affects signaling pathways including NF-ĸB, B-cell receptor, and NOTCH. Treatment with radiation therapy is commonly implemented in localized disease and multiple agents are available for patients with advanced stage disease in need of therapy. Bendamustine with rituximab is the frontline platform associated with higher efficacy. Areas covered: Clinical features, diagnosis, genomics, models enabling risk stratification, treatment options, and future directions. Expert opinion: The lack of consistent genotyping profile in EMZL precludes the development of tissue and circulatory biomarkers for the diagnosis, risk stratification and monitoring of minimal residual disease. Furthermore, the biological heterogeneity observed in extranodal sites associated with overall limited genomic data prevents the testing of druggable pathways aiming for a personalized treatment approach. Future clinical trials should focus on EMZL considering the unique clinical characteristics in the eligibility criteria and response assessment to better inform efficacy of novel agents and delineate sequence of therapies.
【저자키워드】 Diagnosis, risk stratification, lymphomagenesis, treatment approach, extranodal marginal zone lymphoma,