摘要目的 探讨艾滋病相关原发性中枢神经系统淋巴瘤(PCNSL)的临床特征和手术疗效.方法 回顾性分析首都医科大学附属北京地坛医院神经外科2013年1月至2019年2月收治的17例艾滋病相关PCNSL患者的临床资料.17例患者中,12例行肿瘤活组织检查术,其中2例合并梗阻性脑积水的患者同时行脑室-腹腔分流术;另5例行肿瘤切除术,其中3例脑疝患者同时行去骨瓣减压术,l例合并枕骨大孔疝的患者同时行枕大孔减压术,2例合并癫痫的患者同时行责任病灶切除术.对所有患者行电话或门诊随访,电话询问生存状态,门诊复查头颅MRI和进行Karnofsky功能状态评分(KPS).采用Kaplan-Meier曲线比较术后化疗患者与未进行任何治疗患者的生存期.结果 17例患者的手术均成功,术后病理学诊断均为淋巴瘤.合并梗阻性脑积水、脑疝及癫痫的患者,术后症状均明显改善.术后10例患者未行任何治疗,7例行化疗或放疗.17例患者的随访时间为(137.8±7.5)d(13～528 d).随访期间,10例死亡,7例存活.存活的患者中,3例获得影像学随访,显示颅内病灶基本消失或明显缩小;KPS评分分别为60分、60分及80分,均较术前提高(分别为40分、30分、40分).至随访期末,7例患者的生存时间为13～ 528 d.Kaplan-Meier曲线发现,术后化疗患者的生存时间较未进行任何治疗患者的生存时间长,但差异无统计学意义(P＞0.05).结论 艾滋病相关PCNSL的临床特征与免疫功能正常的患者相似,无特殊性.当患者出现严重神经功能障碍,甚至导致脑疝或频发癫痫时,应选择手术治疗,可改善患者的生命质量,同时术后行放、化疗可延长患者的生存期.

abstractsObjective To investigate the clinical characteristics and surgical outcomes of AIDS-related primary central nervous system lymphoma (PCNSL).Methods Clinical data of 17 AIDS-related PCNSL patients admitted to Department of Neurosurgery,Beijing Ditan Hospital Affiliated to Capital Medical University from January 2013 to February 2019 were retrospectively analyzed.Of the 17 patients,12 underwent tumor biopsy and included 2 patients with obstructive hydrocephalus who underwent ventriculoperitoneal shunt.The remaining 5 patients underwent tumor resection and included 3 patients with cerebral hernia who underwent decompressive craniectomy,1 patient with foramen magnum hernia who underwent foramen magnum decompression and 2 patients with epilepsy who underwent responsible lesion resection.All patients were followed up by telephone or outpatient service.Survival status was inquired by telephone and cranial MRI Karnofsky performance status (KPS) were reviewed in outpatient service.Kaplan-Meier curve was used to compare the survival time of patients receiving chemotherapy after operation with that of patients without any treatment.Results All 17 patients underwent successful operation and all of them were diagnosed as lymphoma by pathology.Postoperative symptoms of patients with obstructive hydrocephalus,cerebral hernia or epilepsy were significantly improved.No treatment was given to 11 patients,and 6 patients received chemotherapy or radiotherapy.The follow-up time of 17 patients was 137.8 ± 7.5 (13-528) days.During the follow-up period,10 patients died and 7 survived.Among the surviving patients,3 cases were followed up by imaging showing that the intracranial lesions disappeared or decreased significantly.The KPS scores were 60,60 and 80 points respectively,which were higher than those before operation (40,30 and 40 points respectively).By the end of the follow-up period,the survival time of 7 patients was 13-528 days.By Kaplan-Meier curve,the survival time of patients with post-operative chemotherapy was longer than that of patients without any treatment.However,there was no statistical significance (P ＞ 0.05).Conclusion The clinical features of AIDS-related PCNSL are similar to those of patients with normal immune function.When patients suffer from severe neurological dysfunction even leading to cerebral hernia or frequent epileptic seizures,surgical treatment could improve the quality of life of patients.Meanwhile,postoperative radiotherapy and chemotherapy could prolong the survival time of patients.