原发性中枢神经系统淋巴瘤影像学特征和疗效分析

目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的影像学诊断、治疗及预后,提高临床对PCNSL的认识。方法回顾性分析经术后病理证实的30例免疫正常的原发性中枢神经系统淋巴瘤患者的临床资料。单纯手术8例,手术+化疗14例,手术+化疗+放疗8例。Kaplan-Meier法分析患者生存期。结果PCNSL临床表现主要为颅内压增高和神经功能缺损为主,误诊率高达93.3%。24例PCNSL(占80.0%)MRI明显均匀强化,表现为团块状及结节状强化,或出现具有特异性的"缺口征"、"尖角征"。病理检查均为B细胞淋巴瘤,以弥漫性大B淋巴瘤最多见。Kaplan-Meier分析全组中位生存期32.0个月,2年生存率46.7%。手术+化疗(中位生存期29个月)及手术+化疗+放疗组(34个月)的生存期高于单纯手术组(3个月)。手术+化疗组(64.3%)及手术+化疗+放疗组(62.5%)的2年生存率均高于单纯手术组(0)。放化疗患者肿瘤全切组与次全切组的生存率未见明显的统计学差异(P>0.05)。结论原发性中枢神经系统淋巴瘤影像学缺乏特异性,易误诊;单纯手术治疗不是首选,应尽早采取以放、化疗为主的全身综合治疗。

The imaging features and efficacy analysis of primary central nervous system lymphoma

Objective To investigate the diagnosis,treatment and prognosis of primary central nervous system lymphoma(PCNSL) by analyzing the clinical manifestations,imaging features and pathological characteristics of immunocompetent patients with PCNSL and increase the recognition of PCNSL in clinic.Methods Thirty cases with pathologically confirmed PCNSL were treated in our hospital from Jan.2000 to Nov.2009.Treatments included surgery alone in 8 cases,surgery + chemotherapy in 14 cases,surgery + chemotherapy + radiotherapy in 8 cases.The clinical characteristics,laboratory,imaging,pathological data and the treatments were reviewed retrospectively.SPSS13.0 statistical analysis software was used to analyze the data.The survival curves were calculated according to the Kaplan-Meier method.Results The major symptoms of PCNSL were functional loss of nervous system and high intracranial pressure and 93.3% of patients were misdiagnosed.Obvious homogeneous mass-like or nodular enhancement or the presence of "hilar depression sign" and "angular sign" could be detected in 24 cases(80%) on enhanced MRI.Pathologically,all cases were B-cell lymphoma and most of them were diffuse large B-cell lymphoma.Kaplan-Meier analysis showed that the median survival time(MST) was 32.0 months and the 2-year survival rate was 46.7% across the whole group.Kaplan-Meier analysis also revealed that the survival time of the Group receiving both surgery and chemotherapy or the Group receiving surgery combined with chemotherapy and radiotherapy was longer than the Group receiving surgery only.(MSTsurgery = 3 months,MSTsurgery+ chemotherapy = 29 months,MSTsurgery + chemotherapy + radiotherapy = 34 months).The survival rate of these two Groups was higher than surgery alone group(R 2-year surgery + chemotherapy = 64.3%,R 5-year surgery+ chemotherapy = 14.3%;R 2-year surgery + chemotherapy + radiotherapy = 62.5%,R 5-year surgery+ chemotherapy+ radiotherapy = 25.0%.Both R 2-year and R 5-year of the Group received surgery only were 0).The survival rates were no statistically different between the Group receiving total resection and the Group receiving subtotal resection(P > 0.05).Conclusion PCNSL is a rare highly malignant CNS tumor and often misdiagnosed due to lack of typical imaging features.Surgerical operation alone is not the first treatment option while radiotherapy and chemotherapy-based systemic treatment should be taken as soon as possible