Gerstner Elizabeth, Batchelor Tracy
Massachusetts General Hospital and Harvard Medical School, Department of Neurology, Boston, MA 02114, USA.
Expert Rev Anticancer Ther. 2007 May;7(5):689-700. doi: 10.1586/14737140.7.5.689.
Primary CNS lymphoma, an uncommon form of extranodal non-Hodgkin's lymphoma, has increased in incidence and occurs in both immunocompromised and immunocompetent hosts. Primary CNS lymphoma in immunocompetent patients is associated with unique diagnostic, prognostic and therapeutic issues and the management of this malignancy is different from other forms of extranodal non-Hodgkin's lymphoma. Characteristic imaging features should lead to suspicion of the diagnosis, avoidance of corticosteroids (if possible) and early neurosurgical consultation for stereotactic biopsy. Since primary CNS lymphoma may involve the brain, cerebrospinal fluid and eyes, diagnostic evaluation should include assessment of all of these regions as well as screening for the possibility of occult systemic disease. Resection provides no therapeutic benefit and should be reserved for the rare patient with neurological deterioration due to brain herniation. Whole-brain radiation therapy alone is insufficient for durable tumor control and is associated with a high risk of neurotoxicity in patients over 60 years of age. Neurotoxicity is typically associated with significant cognitive, motor and autonomic dysfunction and has a negative impact on quality of life. Chemotherapy and whole-brain radiation therapy together improve tumor response rates and survival compared with whole-brain radiation therapy alone. Methotrexate-based multiagent chemotherapy without whole-brain radiation therapy is associated with similar tumor response rates and survival compared with regimens that include whole-brain radiation therapy, although controlled trials have not been performed. The risk of neurotoxicity is lower in patients treated with chemotherapy alone. The incidence of HIV-related primary CNS lymphoma has decreased in the era of highly active antiretroviral therapy. Patients with HIV-associated primary CNS lymphoma have a worse prognosis but may respond to highly active antiretroviral therapy, whole-brain radiation therapy or therapies directed against the Epstein-Barr virus.
原发性中枢神经系统淋巴瘤是一种罕见的结外非霍奇金淋巴瘤，其发病率呈上升趋势，可发生于免疫功能低下和免疫功能正常的宿主。免疫功能正常患者的原发性中枢神经系统淋巴瘤存在独特的诊断、预后和治疗问题，这种恶性肿瘤的管理与其他形式的结外非霍奇金淋巴瘤不同。特征性的影像学表现应引起对该诊断的怀疑，避免使用皮质类固醇（如有可能），并尽早进行神经外科会诊以进行立体定向活检。由于原发性中枢神经系统淋巴瘤可能累及脑、脑脊液和眼睛，诊断评估应包括对所有这些区域的评估以及筛查隐匿性全身疾病的可能性。手术切除无治疗益处，应仅用于因脑疝导致神经功能恶化的罕见患者。单纯全脑放射治疗不足以实现持久的肿瘤控制，且与60岁以上患者的神经毒性高风险相关。神经毒性通常与显著的认知、运动和自主神经功能障碍相关，对生活质量有负面影响。与单纯全脑放射治疗相比，化疗联合全脑放射治疗可提高肿瘤缓解率和生存率。与包括全脑放射治疗的方案相比，基于甲氨蝶呤的多药化疗不进行全脑放射治疗的肿瘤缓解率和生存率相似，尽管尚未进行对照试验。单独接受化疗的患者神经毒性风险较低。在高效抗逆转录病毒治疗时代，与HIV相关的原发性中枢神经系统淋巴瘤的发病率有所下降。HIV相关原发性中枢神经系统淋巴瘤患者预后较差，但可能对高效抗逆转录病毒治疗、全脑放射治疗或针对爱泼斯坦-巴尔病毒的治疗有反应。
