Department of Neurology, Medisch Spectrum Twente, Enschede, The Netherlands.
Department of Radiology, Medisch Spectrum Twente, Enschede, The Netherlands.
J Neurol. 2024 May;271(5):2906-2913. doi: 10.1007/s00415-023-12143-w. Epub 2023 Dec 19.
Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin lymphoma (NHL) manifesting in the brain, spinal cord, cerebrospinal fluid and/or eyes, in the absence of systemic manifestations. With an increasing incidence and a 30% 5-year overall survival if promptly treated, timely diagnosis and subsequent treatment is paramount. The typical MRI appearance for PCNSL is a solitary or multiple T2-hypointense, homogeneous gadolinium-enhancing lesion with restricted diffusion. Dexamethasone treatment might compromise and delay the diagnosis. Hallmark of treatment is induction with intravenous high-dose methotrexate consisting polychemotherapy followed by consolidation treatment. Consolidation treatment consists of either whole brain radiotherapy (WBRT) or autologous stem cell transplantation (ASCT). Given the (cognitive) side effects of WBRT, ASCT is increasingly being used as the first choice of treatment.
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（NHL），表现为大脑、脊髓、脑脊液和/或眼睛，没有全身表现。随着发病率的增加，如果及时治疗，5 年总生存率为 30%，因此及时诊断和随后的治疗至关重要。PCNSL 的典型 MRI 表现为单个或多个 T2 低信号、均匀钆增强病变，弥散受限。地塞米松治疗可能会影响和延迟诊断。治疗的标志是诱导静脉内大剂量甲氨蝶呤联合化疗，然后进行巩固治疗。巩固治疗包括全脑放疗（WBRT）或自体干细胞移植（ASCT）。鉴于 WBRT 的（认知）副作用，ASCT 越来越多地被用作首选治疗方法。
