目的探讨原发性中枢神经系统淋巴瘤 (PCNSL)的临床特点。方法对 9例 PCNSL 临床资料进行回顾性分析。结果临床表现主要为头痛、呕吐、视乳头水肿、癫痫及偏瘫等。病灶均位于幕上 ,单发灶 6例 ,多发灶 3例 ,头颅 CT、MRI显示肿块边界多清楚 ,周围脑水肿明显 ,无钙化 ,增强后肿块多均匀一致强化。治疗方法有手术切除 ,术后放疗及化疗。结论 PCNSL临床表现及影像学检查均无特异性 ,术前确诊依赖于脑脊液细胞学检查及立体定向脑活检 ,术后常规放疗加全身及鞘内注药化疗可明显延长生存期 Objective To investigate the clinical features of primary central nervous system lymphoma (PCNSL). Methods The clinical data of 9 cases of PCNSL were retrospectively analyzed. The main clinical manifestations were headache, vomiting, optic disc edema, epilepsy and hemiplegia. The lesions were located in the supratentorial, single lesions in 6 cases, multiple lesions in 3 cases, skull CT, MRI showed more clearly the boundaries of the mass, the surrounding brain edema was obvious, no calcification, and the enhancement of the mass was more uniform and consistent. Treatments include surgical resection, postoperative radiotherapy and chemotherapy. Conclusion The clinical manifestations and imaging findings of PCNSL are non-specific. Preoperative diagnosis is dependent on cerebrospinal fluid cytology and stereotactic brain biopsy. Conventional radiotherapy combined with systemic and intrathecal chemotherapy can significantly prolong survival.