原发性中枢神经系统淋巴瘤属非何杰金氏淋巴瘤。占全部原发性颅内肿瘤的1％弱。近10年来,在非免疫抑制人群中发病率增加了三倍。该肿瘤特别好发于AIDS病人、接受脏器移植行免疫抑制治疗的患者、患有遗传性免疫缺陷者以及其它免疫系统获得性疾病的患者。由于AIDS病人和整体人群中发病例数增多,它可能成为最常见的神经系统肿瘤之一。本文就该肿瘤的命名、分类、病理、临床表现、诊断、治疗和预后作一综述。 Primary central nervous system lymphoma is non-Hodgkin’s lymphoma. It accounts for 1% of all primary intracranial tumors. In the past 10 years, the incidence has increased three-fold in non-immunosuppressed populations. The tumors are particularly prone to AIDS patients, patients receiving immunosuppressive therapy for organ transplants, patients with inherited immunodeficiency, and other immune system-acquired diseases. Due to the increase in the number of cases of AIDS patients and the overall population, it may become one of the most common neurological tumors. This article reviews the nomenclature, classification, pathology, clinical manifestations, diagnosis, treatment and prognosis of this tumor.