Department of Basic Medicine and Biomedical Engineering, School of Stomatology and Medicine, Foshan University, Foshan, Guangdong, China.
Department of Oncology, Affiliated Cancer Hospital & Institute of Guangzhou Medical University, Guangzhou, Guangdong, China.
J Cell Physiol. 2020 Dec;235(12):9143-9165. doi: 10.1002/jcp.29790. Epub 2020 May 18.
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.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的非霍奇金淋巴瘤，具有独特的生物学特征和临床行为，患者预后不佳。大剂量（HD）甲氨蝶呤（HD-MTX）治疗的出现显著改善了 PCNSL 的预后。目前，以 HD-MTX 为基础的化疗方案被认为是一线治疗。PCNSL 对放疗敏感，全脑放疗（WBRT）可巩固化疗反应；然而，WBRT 相关的迟发性神经毒性导致神经认知障碍，尤其是在老年患者中。其他有效的方法包括利妥昔单抗、替莫唑胺和自体造血干细胞移植（ASCT）。此外，针对 PCNSL 的新型药物，如针对 B 细胞受体信号通路的药物，正在进行临床试验。然而，PCNSL 的最佳治疗方法仍未确定。本文综述了过去 5 年中在免疫功能正常的 PCNSL 患者中，手术方法、诱导化疗、放疗、ASCT、挽救治疗和新型治疗方法方面的进展。此外，还根据最近的临床研究结果总结了老年患者和复发/难治性 PCNSL 患者的治疗进展。
