原发性中枢神经系统淋巴瘤研究进展

原发性中枢神经系统淋巴瘤(Primary Central Nervous System Lymphoma,PCNSL)是一种比较罕见的结外淋巴瘤。好发于免疫缺陷的人群中。但近年来在免疫力正常人群中发病率不断增加,目前其发病机制仍有争论。其病理形态与颅外淋巴瘤相似,病理类型一般为中高度恶性非霍奇金淋巴瘤(NHL),多为弥漫型大B细胞来源,来源于T细胞的比较少见。影像学表现为单发或多发的深部脑实质或血管周围病变及脑膜等处的病变。CT平扫呈圆形或卵圆形等密度占位病变,边界相对清楚,周围有水肿带,应与胶质瘤、脑膜瘤、转移瘤鉴别。脑脊液淋巴细胞亚群的流式分析能够对诊断脑膜淋巴瘤有所帮助。在临床表现方面与其它颅内肿瘤无明显差异。放化疗综合治疗有可能提高治愈率。化疗采用以MTX为主的化疗,全脑放疗已被公认为治疗PCNSL的有效手段。放、化疗的顺序在一定程度上可能影响患者的生存期,目前推荐采用先放后化的治疗方法。预后取决于多种因素如年龄、确诊时间、病变部位、肿瘤组织类型、治疗措施的选择、患者有否免疫抑制状态等。

The Advancement in Treatment of Primary Central Nervous System Lymphoma

PCNSL is a rare kind of lymphoma that occurs most frequently in immunodeficient patients.The incidence of PCNSL has increased in recent years in people with normal immune systems,where its mechanism is still unclear.The pathological characteristics of PCNSL are similar to extracranial lymphoma and the degree of malignancy is similar to middle to highly malignant non-Hodgkin's lymphoma.Most PCNSL originate from large B-cells;few of them are from T-cells.PCNSL can be located deep in the brain,around vessels,or in meninges.Single or multiple nodes with clear edema can be seen on a CT scan.PCNSL must be distinguished from glioma,meningioma and brain metastases.Cytological and immunocytochemical studies of the CSF in cases suspected of CNS lymphoma should be performed.The clinical characteristics of PCNSL are no different from other cranial tumors.Combining radiotherapy and chemotherapy may increase the response rate.MTX has been recommended for chemotherapy and whole brain radiotherapy is an effective treatment method.Survival could be affected by the sequence of chemotherapy and radiotherapy:radiotherapy should be performed first.Many factors such as age,position of the tumor,histology,and treatment method may affect the prognosis of PCNSL.