San Raffaele Scientific Institute, Haematology and Bone Marrow Transplantation Unit, Department of Oncology, Milan, Italy.
Expert Opin Pharmacother. 2010 Jun;11(8):1263-76. doi: 10.1517/14656561003767456.
Primary central nervous system lymphomas (PCNSL) are rare but potentially curable tumours. The overall outcome for PCNSL patients is unsatisfactory and several therapeutic questions remain open. Modest progress in outcome reflects difficulties in conducting randomized trials and scarce molecular and biological knowledge.
This review describes conventional and investigational treatments for PCNSL and focuses on the main questions for future clinical trials. PubMed and the authors' own files were utilized for references search. The terms 'PCNSL', 'primary AND CNS lymphoma', and 'CNS AND lymphoma' were used for PubMed queries. All papers published in English before November 2009 were considered.
This review illustrates how the paradigm for PCNSL treatment changed during the 1990s from radiotherapy alone to the establishment of high-dose methotrexate-cytarabine combination as standard approach. We present promising data from Phase II studies and discuss questions for randomized trials. Finally, we offer a 5-year scenario for the management of PCNSL.
TAKE-HOME MESSAGE: The methotrexate-cytarabine combination should currently be considered as the reference treatment for PCNSL. Well-designed randomized trials and biological studies deriving from the use of novel technologies will be crucial to further improve outcome in these patients.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见但可治愈的肿瘤。PCNSL 患者的总体预后并不理想，仍存在一些治疗问题悬而未决。治疗结果的进展甚微反映了进行随机试验的困难以及分子和生物学知识的匮乏。
本文描述了 PCNSL 的常规和研究性治疗方法，并重点介绍了未来临床试验的主要问题。作者检索了 PubMed 和自己的文件，使用了“PCNSL”、“原发性中枢神经系统淋巴瘤”和“中枢神经系统淋巴瘤”作为 PubMed 查询的关键词。所有在 2009 年 11 月之前以英文发表的论文都被纳入考虑。
本文说明了 20 世纪 90 年代 PCNSL 治疗模式如何从单纯放疗转变为大剂量甲氨蝶呤-阿糖胞苷联合治疗作为标准方法。我们展示了来自 II 期研究的有前景的数据，并讨论了随机试验的问题。最后，我们为 PCNSL 的管理提供了一个 5 年的方案。
甲氨蝶呤-阿糖胞苷联合治疗目前应被视为 PCNSL 的标准治疗方法。精心设计的随机试验和从新技术应用中得出的生物学研究对于进一步改善这些患者的预后至关重要。
