原发性中枢神经系统淋巴瘤诊断及治疗进展

Progress in diagnosis and treatment of primary central nervous system lymphoma

目的原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是中枢神经系统肿瘤中非常少见的一类肿瘤,实验室检查指标无特异性,影像学呈多样性,误诊率较高.本研究旨在探究PCNSL诊断及治疗进展.方法应用PubMed、万方和CNKI等中英文数据库,以"原发性中枢神经系统淋巴瘤、诊断、治疗"为检索词,检索2011-01-01-2019-03-31相关文献.纳入标准:与PCNSL诊断治疗相关的文献.排除标准:综述类文献;研究资料缺失.根据纳入和排除标准分析文献42篇.结果PCNSL是中枢神经系统肿瘤中非常少见的一类肿瘤,约占脑肿瘤的2%,发病率约为0.44/10万.PCNSL临床表现主要以高颅压症状为主,但无特异性,诊断常采用多种方式,包括影像学检查、脑脊液检查、病理以及免疫组织化学检查,其中病理检查为金标准,但需通过开颅手术或立体定向获取活检标本.PCNSL的治疗方式主要有手术、放疗和化疗,以及放疗联合化疗等多种方案.虽然PCNSL的最佳治疗方案尚未确定,但大剂量甲氨蝶呤(high dose methotrexate,HD-MTX)化疗目前被认为是新诊断PCNSL的首选治疗方法.结论目前对PCNSL的诊断尚缺乏特异的方法,HD-MTX化疗被认为是PCNSL新诊断的首选治疗方案.

OBJECTIVE Primary central nervous system lymphoma(PCNSL) is a rare type of tumor in the central nervous system tumor. The laboratory examination indexes are not specific and imaging findings are diverse,so the misdiagnosis rate is high. In this study, the diagnosis and treatment progress of PCNSL was reviewed. METHODS Pubmed, Wanfang,CNKI and other Chinese and English databases were used to retrieve the articles from January 1,2011 to March 31,2019 with the keywords of "primary central nervous system lymphoma,diagnosis and treatment". Inclusion criteria was literature related to PCNSL diagnosis and treatment. The exclusion conditions were literature review and lack of research data. Finally,42 articles were included. RESULTS Primary central nervous system lymphoma is a rare type of central nervous system tumors, accounting for about 2% of brain tumor. The incidence is about 0. 44/100 thousand. The clinical manifestations are mainly high intracranial pressure symptoms without specificity. Diagnosis is usually performed in a variety of ways, including imageological examination, cerebrospinal fluid examination, pathological examination and immunohistochemistry. Pathological examination is the golden standard,for which biopsy specimens must be obtained by craniotomy or stereotactic orientation. The treatment of PCNSL mainly includes surgery, radiotherapy, chemotherapy and radiotherapy combined with chemotherapy. High dose methotrexate (HD-MTX) chemotherapy is currently considered as the preferred treatment for newly diagnosed PCNSL although the optimal treatment regimen has not been established. CONCLUSIONS At present, there is no specific method for the diagnosis of PCNSL. HD-MTX chemotherapy is currently considered as the preferred treatment for newly diagnosed PCNSL.