摘要
目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的临床与影像学特点,治疗与预后。方法对40例病理证实的 PCNSL 的临床与影像学表现、治疗和预后情况进行回顾性分析。结果PCNSL 好发于50～60岁男性,急性或亚急性起病。肿瘤单发者占75.0%,多发者占25.0%,多位于脑脊液循环通路附近,即深部结构近脑室(57.5%)或靠近皮质的浅表部位(32.5%)。病理均为 B 细胞型,有特征性的"血管周围淋巴套"和"星空现象"。临床不具有特异性。头颅 MRI 多为 T_1低信号,T_2高信号,界限清晰,水肿明显,显著均匀强化占67.6%,厚壁环形强化占13.5%。采用大剂量甲氨蝶呤(HDMTX)化疗者,存活率(75.0%)较未采用者(48.1%)高。平均生存时间14.8个月,复发后69.2%的患者存活不足6个月。结论 PCNSL 临床表现无特异性,确诊需靠穿刺活体组织检查。治疗首选 HDMTX 为基础的综合化疗,预后差。
Objective To explore the clinical and radiological features, treatment strategy and prognosis of primary central nervous system lymphoma （PCNSL）. Methods Forty pathologically established PCNSL patients were enrolled for retrospective analysis of clinical, radiological and pathological presentations, treatment strategies and survival periods. Results PCNSL was more common in males of 50-60 years old with an acute or subacute onset, seventy-five percent of the patients presented with solitary lesions and 25% muhifocal lesions. Most of the lymphomas were adjacent to the cerebral spinal fluid pathway either in deep structures near ventricle （57.5%） or superficial structures near cortex （32. 5% ）. Almost all were of B cell type, with characteristic phenomenon of ＂cuff＂ of tumor cells surrounding the vessels and ＂stars in sky ＂. The clinical manifestations were nonspecific. Most of the tumors were hypointense on T1 -weighed and hyperintense on T2-weighed with clear edge and obvious edema, sixty-seven point six percent of the tumors enhanced homogeneously and 13.5% had ring enhancing phenomenon. Patients undergoing high dose methotrexate （HDMTX） based chemotherapy had a higher survival rate （75.0%） than those treated without HDMTX. The mean survival period was only 14. 8 months. After relapse, 69. 2% of the patients survived less than 6 month. Conclusions PCNSL has nonspecific clinical manifestations, whose diagnosis relies on stereotactic biopsy. HDMTX based chemotherapy is the first treatment of choice, and the prognosis of PCNSL is poor with a mean survival period of 14. 8 months.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2007年第10期683-686,共4页
Chinese Journal of Neurology
关键词
中枢神经系统肿瘤
淋巴瘤
甲氨蝶呤
Central nervous system neoplasms
Lymphoma
Methotrexate
