Gonzalez-Aguilar A, Houillier C, Soussain C, Hoang-Xuan K
UPMC, Service de neurologie Mazarin, groupe hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, Paris cedex 13, France.
Rev Neurol (Paris). 2011 Oct;167(10):721-8. doi: 10.1016/j.neurol.2011.08.004. Epub 2011 Sep 8.
Primary CNS lymphoma is the malignant brain tumor whose prognosis has improved the most the two past decades. The majority of the patients achieve a complete remission with treatment and a substantial minority may hope to be cured. The treatment includes high-dose methotrexate polychemotherapy combined or not with whole brain radiotherapy (WBRT). Elderly patients who are exposed to a high risk of treatment induced neurotoxicity need a specific management avoiding or defering WBRT. In young patients, the main questions concerning the treatment are the role of consolidation WBRT and intensive chemotherapy with peripheral blood stem cell rescue in the initial treatment of PCNSL. As recently shown, national and international collaborative efforts make now possible randomized trials for this rare disease, which would contribute to better define the treatment strategy. New insights in PCNSL tumorigenesis would help to better understand the heterogeneity of outcome and to develop efficient targeted therapies.
原发性中枢神经系统淋巴瘤是一种恶性脑肿瘤，在过去二十年中其预后改善最为显著。大多数患者通过治疗可实现完全缓解，少数患者有望治愈。治疗方法包括高剂量甲氨蝶呤联合化疗，可联合或不联合全脑放疗（WBRT）。面临治疗诱导神经毒性高风险的老年患者需要特殊管理，避免或推迟WBRT。在年轻患者中，关于治疗的主要问题是巩固性WBRT的作用以及在原发性中枢神经系统淋巴瘤初始治疗中采用外周血干细胞救援的强化化疗。正如最近所显示的，国家和国际合作努力现在使得针对这种罕见疾病进行随机试验成为可能，这将有助于更好地确定治疗策略。原发性中枢神经系统淋巴瘤肿瘤发生的新见解将有助于更好地理解结果的异质性并开发有效的靶向治疗方法。
