【作者】 刘群； 李鹏； 王鹏； 朴颖哲； 王晓光； 李文良；

【Author】 Liu Qun Li Peng Wang Peng PiaoYing-Zhe Wang Xiao-Guang Li Wen-Liang Department of Neurosurgery, AfRiiated Cancer Hospital, Tianjin Medical University, Tianjin 300060. Department of Neurosurgery, Affiliated Cancer Hospitol, Tianjin Medical University, Tianjin 300060

【机构】 天津医科大学附属肿瘤医院颅脑肿瘤科；

【摘要】 目的:原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是一种罕见的发生于中枢神经系统的非霍奇金淋巴瘤,病变局限于中枢神经系统内,不伴有其它系统的播散,近些年发病率有所上升,且预后不佳。本文探讨原发性中枢神经系统淋巴瘤的临床、影像学特点和治疗方法。方法:回顾性分析我院收治的经病理证实的22例PCNSL患者的临床资料和治疗效果。2002年11月以前采用以手术、CHOP方案为主化疗、联合全脑放疗的治疗方法,2002年12月以后采用以HD—MTX为主的联合化疗并结合全脑放疗的治疗方法。结果:22例PENSL患者中,男性 10例,女性12例,中位年龄49岁。19例(86．4％)主要表现为颅内高压,单病灶18例,多病灶4例。CT平扫表现为类圆形,近等密度,边界较清,周围中度水肿。MRI T wI为等低信号,T wI为等高信号,边界较清,中度水肿,增强后强化明显且较均匀。22例组织病理标本中来源于手术切除的20 例,立体定向活检2例。病理检查为B细胞来源者占77．2％ (17／22),其中14例为弥漫性B大细胞淋巴瘤。依国际工作分类(WF),中度恶性占59．1％(13／22)。22例中位随访期 13．5个月(1～84个月),Kaplan—Meier分析总中位生存期26 个月,2年生存率45．7％;HD—MTX联合放疗组患者完全缓解率61．1％,中位生存期26个月以上,,2年生存率75．1％, 疗效明显优于非HD—MTX联合放疗组。结论:PCNSL多发于中老年人,颅内高压为主要表现,B细胞亚型占绝对优势。影像学检查有一定的特殊性,多见肿瘤边界清楚,质地较均匀,周围水肿多不严重。CT平扫近等密度,MRI T wI为等低信号,T wI为等高信号,边界较清,增强后强化明显且较均匀。影像学检查考虑PCNSL的不应手术,应先行立体定向活检,若诊断为PENSL不应手术治疗,应行HD—MTX 为主联合化疗并结合全脑放射治疗,同时应行腰穿细胞学检查,对于阳性的病人可在放化疗的同时行鞘注MTX治疗可以改善预后,阴性的病人鞘注MTX治疗可能也有一定的效果。对于老年病人或无预后不良因素的病人可适当减少放疗剂量并增加化疗剂量防止远期神经系统毒性。具体治疗方法的剂量和方式还需要进一步前瞻性研究来确定。更多还原

【Abstract】 Objective: Primary central nervous system lymphoma(PCNSL)is a type of non - Hodgkin’ S lymphoma(NHL), which originates in the central nervous system and never spread outside. Over the past decade, the incidence of PCNSL has increased. But the prognosis are still poor for most of the patients, The aim of this research is to analyze the clinical and neuroimage characteristics of primary centralnervous system lymphoma and explore the methods of treatment Methods: Twenty-two patients with PCNSL treated in our hospital from 1989 to 2002 were retrospectively reviewed. Before nov.2002,sur-gery and CHOP with or without whole brain radiotherapy (WBRT) was employed; after then high-dose MTX-based chemotherapy with WBRT was employed. Results: Of 28 patients, 18 men and 10 women with a median age of 52 year. multiple lesions were reported in 18 patients. 4 cases were single lesion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain typically show unique or multiple periventricular, uniformly enhancing lesions with minimal mass effect, clear margins and moderate perifocal edema. 77.2% (17/22) of the patients were diagnosed as having B-cel origin,its main type being difuse large cell lymphoma. According to international working formulation(WF),moderate-grade of his-topathology was observed in 59.1%(13/22). Kaplan-meier test showed that the median overall survival time was 26 months, and the 2-year survival rate was 45.7%. The complete response rate of the 18 patients who received high-dose MTX-based chemotherapy plus WBRT was 61.1%, the median survival time was more than 26 months, and the 2-years survival rate was 75.1%. The efficacy of high-dose MTX-based chemotherapy plus WBRT was better than that of CHOP plus WBRT. Conclusion: PCNSL often occurs in middle-aged and aged patients, with intrac-ranial hypertension as the main clinical manifestation. B-cell lymphoma is the predominant subtype. Characteristic imaging features of PCNSL are uniformly enhancing lesions with minimal mass effect, clear margins and moderate perifocal edema. Surgery is ineffective for PCNSL. Resection provides no therapeutic benefit and should be reserved for the rare patient with neurologic deterioration due to brain herniation. PCNSL suspected by neuroimaging must be confirmed via stereotactic biopsy. High-dose MTX-based chemotherapy plus WBRT is efficient and feasible for PCNSL. Furthermore, intrathecal administration of MTX may be useful for the positive cases of cere-brospinal fluid cellular examination, even for the negative cases. For the patients with good prognostic factors, reduction of radiotherapy dose could reduce the neurotoxicity. Future trials should attempt to define the best polychemotherapy regimen for PCNSL.更多还原