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目的:总结原发性中枢神经系统淋巴瘤(PCNSL)的临床特点、诊治方案及临床疗效。方法:回顾收治的21例PCNSL患者,均为病理确诊的B细胞来源非霍奇金淋巴瘤,其中5例接受单纯放疗,16例接受放化疗。对病理学检查、影像学表现、治疗及预后进行总结分析。结果:PCNSL以中老年人多见,发病急,病程短,病情进展快。临床表现复杂,颅内高压为主要表现之一。CT、MR增强扫描病灶多呈均匀明显强化,可单发或多发。21例患者中位生存时间22个月,1、3和5年生存率分别为76.2%、28.6%和4.76%。放化疗疗效优于单纯放疗,P=0.029。结论:PCNSL临床表现多样,影像学缺乏特异性,极易误诊,确诊需要依靠病理学检查,其最佳治疗方案是三维立体定向穿刺活检加放疗、化疗的联合治疗。PCNSL侵袭性强,生存期短,其预后主要与发病年龄、多灶性和体力状况有关。
Objective: To summarize the clinical features, diagnosis and treatment of primary central nervous system lymphoma (PCNSL) and its clinical efficacy. Methods: A retrospective study of 21 patients with PCNSL were pathologically diagnosed B-cell non-Hodgkin’s lymphoma. Of these, 5 received radiotherapy alone and 16 received chemoradiation. The pathological examination, imaging findings, treatment and prognosis were analyzed. Results: PCNSL was more common in middle-aged and elderly patients, with acute onset, short course and rapid progression. Clinical manifestations of complex, intracranial hypertension as one of the main performance. CT, MR enhanced scanning lesions were mostly significantly enhanced, can be single or multiple. The median survival time of 21 patients was 22 months and the 1,3 and 5 year survival rates were 76.2%, 28.6% and 4.76%, respectively. Radiotherapy and chemotherapy is superior to radiotherapy alone, P = 0.029. Conclusion: The clinical manifestations of PCNSL are diverse, the imaging is lack of specificity and easily misdiagnosed. The diagnosis depends on the pathological examination. The best treatment is the combination of three-dimensional stereotactic biopsy plus radiotherapy and chemotherapy. PCNSL aggressive, short survival, the prognosis and the age of onset, multifocal and physical status.