Department of Haematology and Oncology, Charité-Campus Benjamin Franklin, University Medicine Berlin, Hindenburgdamm 30, 12200 Berlin, Germany.
Nat Rev Neurol. 2013 Jun;9(6):317-27. doi: 10.1038/nrneurol.2013.83. Epub 2013 May 14.
Primary CNS lymphoma (PCNSL) is a rare lymphoma that is confined to the CNS, with low tendency for systemic dissemination and a relatively aggressive course. Outcome in patients with PCNSL is often poor. Owing to its low incidence, current knowledge about optimal treatment of PCNSL is fragmentary. Chemotherapy regimens based on high-dose methotrexate are currently standard treatment for all patients with PCNSL who can tolerate such drugs. Whole-brain radiotherapy alone can lead to remission in up to 90% of patients, but often results in poor long-term disease control when given alone, and in delayed neurotoxicity when given after high-dose methotrexate. In this Review, we describe current approaches to diagnosis and treatment of PCNSL, and discuss novel therapeutic approaches that are currently in development, such as the use of rituximab and high-dose chemotherapy followed by autologous stem-cell transplantation. The possible use of intrathecal and intraventricular chemotherapy, optimal salvage treatment, and specific treatment approaches in elderly, paediatric and immunocompromised patients, are also considered.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的淋巴瘤，局限于中枢神经系统，全身播散倾向低，病程相对侵袭性强。PCNSL 患者的预后通常较差。由于发病率低，目前关于 PCNSL 的最佳治疗方法的知识还很零碎。基于大剂量甲氨蝶呤的化疗方案是目前所有能耐受此类药物的 PCNSL 患者的标准治疗方法。单纯全脑放疗可使高达 90%的患者获得缓解，但单独使用时往往导致长期疾病控制不佳，在大剂量甲氨蝶呤治疗后使用时则会导致迟发性神经毒性。在这篇综述中，我们描述了 PCNSL 的目前诊断和治疗方法，并讨论了目前正在开发的新的治疗方法，如利妥昔单抗和大剂量化疗后自体干细胞移植的应用。还考虑了鞘内和脑室化疗的可能应用、最佳挽救治疗以及老年、儿科和免疫功能低下患者的特定治疗方法。
