大约30%的淋巴瘤发生在淋巴结、脾脏或骨髓之外，而异型淋巴瘤的发病率在过去十年中一直在上升。虽然传统的化疗和放疗可以改善某些患者的生存结果，但异型淋巴瘤患者的预后仍然不尽人意。不同解剖部位的异型淋巴瘤往往具有不同的细胞起源、发病机制和临床表现，这对它们的诊断和治疗有着重要影响。因此，有必要全面概述异型淋巴瘤的发病机制、诊断和治疗进展，并针对不同的解剖部位进行具体综述。本综述总结了异型淋巴瘤发展中常见的关键信号通路及干预治疗的最新进展。此外，本文还提供了胃黏膜相关淋巴组织（MALT）淋巴瘤、真菌样肿瘤（MF）、自然杀伤/T细胞淋巴瘤（鼻型，NKTCL-NT）和原发中枢神经系统淋巴瘤（PCNSL）等常见异型淋巴瘤的发病机制、诊断和治疗策略的见解。此外，由于PCNSL是预后最差的异型淋巴瘤之一，本文特别总结了预后指标，并讨论了与其临床应用相关的挑战和机遇。本综述的目的是帮助临床医生和研究人员了解异型淋巴瘤的现状，使他们能够做出明智的临床决策，有助于改善患者的预后。© 2023. 四川国际医学交流与促进协会。

Approximately 30% of lymphomas occur outside the lymph nodes, spleen, or bone marrow, and the incidence of extranodal lymphoma has been rising in the past decade. While traditional chemotherapy and radiation therapy can improve survival outcomes for certain patients, the prognosis for extranodal lymphoma patients remains unsatisfactory. Extranodal lymphomas in different anatomical sites often have distinct cellular origins, pathogenic mechanisms, and clinical manifestations, significantly influencing their diagnosis and treatment. Therefore, it is necessary to provide a comprehensive summary of the pathogenesis, diagnosis, and treatment progress of extranodal lymphoma overall and specifically for different anatomical sites. This review summarizes the current progress in the common key signaling pathways in the development of extranodal lymphomas and intervention therapy. Furthermore, it provides insights into the pathogenesis, diagnosis, and treatment strategies of common extranodal lymphomas, including gastric mucosa-associated lymphoid tissue (MALT) lymphoma, mycosis fungoides (MF), natural killer/T-cell lymphoma (nasal type, NKTCL-NT), and primary central nervous system lymphoma (PCNSL). Additionally, as PCNSL is one of the extranodal lymphomas with the worst prognosis, this review specifically summarizes prognostic indicators and discusses the challenges and opportunities related to its clinical applications. The aim of this review is to assist clinical physicians and researchers in understanding the current status of extranodal lymphomas, enabling them to make informed clinical decisions that contribute to improving patient prognosis.© 2023. Sichuan International Medical Exchange & Promotion Association.