Kaddu-Mulindwa Dominic, Thurner Lorenz, Christofyllakis Konstantinos, Bewarder Moritz, Kos Igor Age
Department of Internal Medicine I, Hematology and Oncology, Rheumatology and Clinical Immunology, Saarland University Medical Center, 66424 Homburg, Germany.
Cancers (Basel). 2022 Jun 19;14(12):3019. doi: 10.3390/cancers14123019.
Extranodal marginal zone lymphoma (EMZL) encompasses a subgroup of non-Hodgkin lymphomas that often present with localized involvement and may manifest in a diversity of organs and tissues. EMZL pathogenesis is in some cases linked to chronic inflammation/infection, which may impose additional diagnostic and clinical challenges. The most studied and established connection is the presence of in gastric EMZL. Due to its heterogeneity of presentation and intricate pathological features, treatment can be complex, and staging systems are decisive for the choice of therapy. Nevertheless, there is no consensus regarding the most suitable staging system, and recommendations vary among different countries. As a rule of thumb, in limited stages, a local therapy with surgery or radiation is the preferred option, and it is potentially curative. Of note, eradicating the causal agent may be an important step of treatment, especially in gastric EMZL, in which Helicobacter pylori eradication remains the first-line therapy for the majority of patients. In patients with more advanced stages, watch-and-wait is a valuable option, especially amongst those without clear indications for systemic therapy, and it may be carried on for several years. If watch-and-wait is not an option, systemic therapy may be needed. Even though several agents have been tested as monotherapy or in combination in recent years, there is no consensus regarding the first-line therapy, and decisions can vary depending on individual factors, such as age, clinical performance and stage. This review aims to discuss the several aspects of EMZL, including genetic milieu, pathogenesis and staging systems, that may influence the choice of therapy. In addition, we present a summary of evidence of several systemic therapies, compare different recommendations worldwide and discuss future perspectives and novelties in its therapy.
结外边缘区淋巴瘤（EMZL）是一组非霍奇金淋巴瘤，常表现为局限性受累，可累及多种器官和组织。在某些情况下，EMZL的发病机制与慢性炎症/感染有关，这可能给诊断和临床带来额外挑战。研究最多且已明确的关联是胃EMZL中存在[此处原文缺失相关内容]。由于其临床表现的异质性和复杂的病理特征，治疗可能较为复杂，分期系统对治疗方案的选择起决定性作用。然而，对于最合适的分期系统尚无共识，不同国家的推荐也有所不同。一般来说，在局限性阶段，手术或放疗等局部治疗是首选方案，且可能治愈。值得注意的是，根除病因可能是治疗的重要一步，尤其是在胃EMZL中，根除幽门螺杆菌仍是大多数患者的一线治疗方法。对于更晚期的患者，观察等待是一种有价值的选择，尤其是那些没有明确全身治疗指征的患者，这种观察等待可以持续数年。如果观察等待不可行，则可能需要全身治疗。尽管近年来有几种药物已作为单一疗法或联合疗法进行了测试，但对于一线治疗尚无共识，治疗决策可能因个体因素而异，如年龄、临床表现和分期。本综述旨在讨论EMZL的几个方面，包括遗传背景、发病机制和分期系统，这些方面可能会影响治疗方案的选择。此外，我们还总结了几种全身治疗的证据，比较了全球不同的推荐，并讨论了其治疗的未来前景和新进展。
