Bessell Eric M, Hoang-Xuan Khe, Ferreri Andres J M, Reni Michele
Department of Clinical Oncology, Nottingham City Hospital, Hucknall Road, Nottingham, UK.
Eur J Cancer. 2007 May;43(7):1141-52. doi: 10.1016/j.ejca.2006.12.011. Epub 2007 Apr 12.
This review was produced from the workshop on primary central nervous system lymphoma (PCNSL) at the European Cancer Conference (ECCO 13) in Paris in 2005. It covers the presentation and biological features of the disease (Professor Khe Hoang-Xuan). The role of chemotherapy, including the management of intraocular lymphoma and the use of high dose chemotherapy followed by autologous stem cell transplantation for PCNSL, is discussed (Dr. Andres Ferreri) as well as controversies in the use of whole brain radiotherapy (WBRT) after chemotherapy (Dr. Michele Reni). The topics covered with discussants at the workshop are also summarised.
The imaging of the brain and the histopathology including detailed immunohistochemistry is of vital importance in making an accurate diagnosis of the disease and understanding the extent of spread of the disease in the CNS. The importance of high dose methotrexate (HDMTX; dose > or = 1g/m(2)), as the most active drug in the treatment of PCNSL, is stressed. The authors recommend that HDMTX alone or in combination with other active chemotherapy agents should be used to treat PCNSL followed by whole brain radiotherapy (WBRT) unless contraindicated because of the advanced age of the patient and existing cognitive impairment. Only published protocols should be used unless the patient is to be offered a trial that has either national or international support. Baseline neuropsychological tests should be carried out before treatment and repeated during and after treatment. The risks of cognitive impairment associated with the disease, with methotrexate - containing chemotherapy and with whole brain radiotherapy should be explained to patients and relatives when obtaining informed consent. Long-term survival, with current treatment regimes, is possible with PCNSL but this appears limited to patients less than 60 years of age at presentation (mostly patients less than 50 years of age).
本综述源自2005年在巴黎召开的欧洲癌症大会（ECCO 13）上关于原发性中枢神经系统淋巴瘤（PCNSL）的研讨会。它涵盖了该疾病的临床表现和生物学特征（何黄宣教授）。讨论了化疗的作用，包括眼内淋巴瘤的治疗以及高剂量化疗后自体干细胞移植在PCNSL中的应用（安德烈斯·费雷里博士），以及化疗后全脑放疗（WBRT）使用中的争议（米凯莱·雷尼博士）。还总结了研讨会上与讨论者探讨的主题。
脑部影像学检查和组织病理学检查，包括详细的免疫组化，对于准确诊断该疾病以及了解疾病在中枢神经系统中的扩散程度至关重要。强调了高剂量甲氨蝶呤（HDMTX；剂量≥1g/m²）作为治疗PCNSL最有效的药物的重要性。作者建议，除非因患者年龄较大和存在认知障碍而禁忌，否则应使用HDMTX单独或与其他活性化疗药物联合治疗PCNSL，随后进行全脑放疗（WBRT）。除非患者参与有国家或国际支持的试验，否则应仅使用已发表的方案。治疗前应进行基线神经心理学测试，并在治疗期间和治疗后重复进行。在获得知情同意时，应向患者及其亲属解释与该疾病、含甲氨蝶呤化疗以及全脑放疗相关的认知障碍风险。采用当前的治疗方案，PCNSL患者有可能实现长期生存，但这似乎仅限于初诊时年龄小于60岁的患者（大多数为年龄小于50岁的患者）。
