Department of Neurology and Neurological Sciences, Stanford University School of Medicine, 875 Blake Wilbur, MC 6510, Stanford, Palo Alto, CA, 94305, USA.
Curr Neurol Neurosci Rep. 2024 Nov 25;25(1):5. doi: 10.1007/s11910-024-01389-0.
Optimal initial management can have a significant impact in long-term outcome in primary CNS lymphoma. This article reviews recent advances and the state of the field.
Genomic analysis of CSF cell-free DNA has emerged as a new diagnostic tool for PCNSL. Treatment options have likewise evolved, with mature data from first-line chemotherapy-based prospective trials disclosing excellent results in younger (< 60-65) patients, with a cure achieved in a majority. However, results in older patients remain dismal, with several new salvage options under investigation including BTK pathway-targeted therapies, and CAR-T cell treatments. Meanwhile, low-dose radiation has emerged as an additional alternative for consolidation therapy. For younger PCNSL patients, the goal of treatment is now a cure, with the next frontier being the development of therapies affording optimized neurocognitive outcome and lower toxicity. Treatment for older patients remains however an unmet need, with several promising clinical trials ongoing.
原发性中枢神经系统淋巴瘤(PCNSL)的初始最佳治疗方案对长期预后有显著影响。本文就 PCNSL 的最新进展和研究现状进行综述。
脑脊液游离 DNA 的基因组分析已成为 PCNSL 的一种新的诊断工具。治疗方案也在不断发展,一线基于化疗的前瞻性试验的成熟数据显示,年轻患者(<60-65 岁)的治疗效果极好,多数患者得到治愈。然而,老年患者的治疗效果仍不容乐观,目前正在研究多种新的挽救性治疗方案,包括 BTK 通路靶向治疗和 CAR-T 细胞治疗。同时,低剂量放疗也成为巩固治疗的另一种选择。对于年轻的 PCNSL 患者,目前的治疗目标是治愈,下一前沿是开发能够优化神经认知结局和降低毒性的治疗方法。然而,对于老年患者的治疗仍然是一个未满足的需求,目前正在进行多项有前途的临床试验。