文章：

原发性中枢神经系统淋巴瘤（PCNSL）的临床病理与基因组特征及治疗前景综述

The Landscape of Primary Central Nervous System Lymphoma (PCNSL): Clinicopathologic and Genomic Characteristics and Therapeutic Perspectives

原文发布日期：4 September 2025

DOI: 10.3390/cancers17172909

类型: Article

开放获取: 是

英文摘要：

Primary central nervous system lymphoma (PCNSL) is a rare extra-nodal non-Hodgkin lymphoma confined to the central nervous system. The cancer biology of PCNSL remains incomplete and is often associated with genetic aberrations with abnormal signaling pathways, cell differentiation, regulation of epigenetic modification, and the tumor microenvironment. Stereotactic brain biopsy remains the gold standard for the diagnosis of PCNSL. For patients ill-suited for biopsy, MYD88 and IL-10 may be important biomarkers to diagnose PCNSL. High-dose methotrexate-based polychemotherapy is currently the standard induction treatment for PCNSL, followed by consolidation treatments including autologous stem cell transplant and whole-brain radiotherapy. Some studies suggest that low-dose lenalidomide is recommended as a maintenance therapy for PCNSL. Currently, relapse rates of PCNSL range from 25 to 50% with poor prognosis. Insight research is necessary to identify novel targeted treatments to improve outcomes in relapsed/refractory disease, such as immunomodulatory drugs, immune checkpoint inhibitors, signaling pathway inhibitors, and chimeric antigen receptor T-cell therapy.

摘要翻译： 

原发性中枢神经系统淋巴瘤（PCNSL）是一种局限于中枢神经系统的罕见结外非霍奇金淋巴瘤。其肿瘤生物学机制尚未完全阐明，通常与遗传异常相关，涉及信号通路紊乱、细胞分化异常、表观遗传修饰调控失调以及肿瘤微环境异常。立体定向脑活检仍是诊断PCNSL的金标准。对于不适宜活检的患者，MYD88和IL-10可能成为诊断PCNSL的重要生物标志物。以大剂量甲氨蝶呤为基础的联合化疗是目前PCNSL的标准诱导治疗方案，后续需进行巩固治疗，包括自体干细胞移植和全脑放疗。部分研究表明，低剂量来那度胺可作为PCNSL的维持治疗推荐方案。目前PCNSL复发率在25%至50%之间，预后较差。需深入开展研究以探索新型靶向治疗，改善复发/难治性患者的临床结局，例如免疫调节药物、免疫检查点抑制剂、信号通路抑制剂以及嵌合抗原受体T细胞疗法等。

原文链接：

The Landscape of Primary Central Nervous System Lymphoma (PCNSL): Clinicopathologic and Genomic Characteristics and Therapeutic Perspectives