Central nervous system lymphoma (CNSL) is a rare form of extranodal non-Hodgkin lymphoma. Central nervous system lymphoma can be primary (isolated to the central nervous space) or secondary in the setting of systemic disease. Treatment of CNSL has improved since the introduction of high-dose methotrexate and aggressive consolidation regimens. However, results after treatment are durable in only half of patients, and long-term survivors may experience late neurotoxicity, impacting quality of life. Given the rarity of this disease, few randomized prospective trials exist. This leaves many questions unanswered regarding optimal first-line and salvage treatments. Recent advances in the knowledge of pathophysiology of CNSL will hopefully help the development of future treatments. This review gives an overview of the epidemiology, pathophysiology, clinical presentation, diagnosis, and treatment of immunocompetent patients with CNSL.

中枢神经系统淋巴瘤（CNSL）是结外型非霍奇金淋巴瘤的一种罕见形式。中枢神经系统淋巴瘤可以是原发性（隔离于中枢神经空间），也可以继发于全身性疾病。自引入大剂量甲氨蝶呤以来，对CNSL的治疗已有所改善和积极的合并方案。但是，只有一半的患者接受治疗后的结果持久，长期幸存者可能会出现晚期神经毒性，从而影响生活质量。考虑到这种疾病的罕见性，很少有随机的前瞻性试验。这就留下了许多关于最佳一线和抢救治疗的问题。CNSL病理生理学知识的最新进展有望帮助将来治疗的发展。这篇综述概述了具有免疫功能的CNSL患者的流行病学，病理生理学，临床表现，诊断和治疗。