原发性中枢神经系统淋巴瘤是仅累及中枢神经系统和眼睛的一种非霍奇金淋巴瘤,临床少见,发病机制仍不明确。临床常表现为认知和行为改变、颅压高、局灶神经功能缺失。该病术前诊断困难,影像学检查结合脑脊液细胞学、免疫组织化学、流式细胞技术及单克隆基因重排技术可为术前诊断提供依据,病理诊断仍是金标准。最佳治疗方案尚未确定,放、化疗联合治疗和单纯化疗仍是研究重点。随着总体生存时间的改善,治疗的远期神经损害更加突出。 Primary central nervous system lymphoma is a non-Hodgkin’s lymphoma involving only the central nervous system and the eye. It is rare in clinical practice and its pathogenesis remains unclear. Common clinical manifestations of cognitive and behavioral changes, high intracranial pressure, focal neurological deficits. The preoperative diagnosis is difficult, and imaging combined with cerebrospinal fluid cytology, immunohistochemistry, flow cytometry and monoclonal gene rearrangement technology can provide the basis for preoperative diagnosis, pathological diagnosis is still the gold standard. The best treatment has not been determined, radiotherapy and chemotherapy combined with chemotherapy and chemotherapy alone is still the focus of research. As the overall survival time improved, the treatment of long-term neurological damage more prominent.