Primary central nervous system lymphoma (PCNSL), a rare variant of non‐Hodgkin's lymphoma, is characterized by distinct biological characteristics and clinical behaviors, and patient prognosis is not satisfactory. The advent of high‐dose (HD) methotrexate (HD‐MTX) therapy has significantly improved PCNSL prognosis. Currently, HD‐MTX‐based chemotherapy regimens are recognized as first‐line treatment. PCNSL is sensitive to radiotherapy, and whole‐brain radiotherapy (WBRT) can consolidate response to chemotherapy; however, WBRT‐associated delayed neurotoxicity leads to neurocognitive impairment, especially in elderly patients. Other effective approaches include rituximab, temozolomide, and autologous stem‐cell transplantation (ASCT). In addition, new drugs against PCNSL such as those targeting the B‐cell receptor signaling pathway, are undergoing clinical trials. However, optimal therapeutic approaches in PCNSL remain undefined. This review provides an overview of advances in surgical approaches, induction chemotherapy, radiotherapy, ASCT, salvage treatments, and novel therapeutic approaches in immunocompetent patients with PCNSL in the past 5 years. Additionally, therapeutic progress in elderly patients and in those with relapsed/refractory PCNSL is also summarized based on the outcomes of recent clinical studies.

中文翻译：

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原发性中枢神经系统淋巴瘤治疗的进展和挑战。

原发性中枢神经系统淋巴瘤（PCNSL）是非霍奇金淋巴瘤的一种罕见变体，其特点是具有明显的生物学特征和临床行为，患者的预后并不令人满意。大剂量（HD）甲氨蝶呤（HD-MTX）治疗的出现显着改善了PCNSL的预后。目前，基于HD-MTX的化疗方案被认为是一线治疗。PCNSL对放射疗法敏感，全脑放射疗法（WBRT）可以巩固对化学疗法的反应；然而，WBRT相关的迟发性神经毒性导致神经认知障碍，特别是在老年患者中。其他有效方法包括利妥昔单抗，替莫唑胺和自体干细胞移植（ASCT）。此外，针对PCNSL的新药，例如针对B细胞受体信号传导途径的药物，正在接受临床试验。但是，PCNSL中的最佳治疗方法仍然不确定。这篇综述概述了过去5年中对具有免疫功能的PCNSL患者的手术方法，诱导化学疗法，放射疗法，ASCT，抢救治疗以及新型治疗方法的进展。此外，根据最近的临床研究结果，还总结了老年患者和复发/难治性PCNSL患者的治疗进展。