颅内肿瘤样脱髓鞘病与原发性中枢神经系统淋巴瘤临床误诊病理分析  

Clinicopathological analysis of misdiagnosed tumefactive demyelinating lesions and primary central nervous system lymphoma

作　　者：孙辰婧[1,2] 洪柳[3] 刘建国[2] 路平[3] 桂秋萍[4] 戚晓昆[2] 

机构地区：[1]第二军医大学海军临床医学院神经内科,北京100048 [2]解放军海军总医院神经内科 [3]解放军海军总医院病理科 [4]解放军总医院病理科

出　　处：《中华神经科杂志》2015年第9期757-762,共6页Chinese Journal of Neurology

基　　金：首都医学发展科研基金项目（2009-2054）;海军总医院归国人员创新技术项目（GGCX201205）

摘　　要：目的 回顾性研究肿瘤样脱髓鞘病（TDL）与原发性中枢神经系统淋巴瘤（PCNSL）互相误诊的病例临床影像及病理学特点,总结经验以期提高对TDL与PCNSL的鉴别诊断能力.方法 对2002-2014年收住我院的曾被互相误诊的经病理证实的4例TDL和9例PCNSL进行临床症候、头颅CT、头颅MRI平扫与增强扫描等影像学特点及多项病理组织学特点的对比分析.结果 （1）影像学特点：TDL患者头颅CT均表现为低密度,不同病程其MRI增强扫描表现多样（环形强化3例,点灶状强化l例）;PCNSL患者头颅CT病灶高密度5例,低密度3例,等密度1例,头颅MRI增强均呈均匀一致的强化.（2）病理特点：TDL患者可见大量髓鞘脱失,而少数轴索断裂损伤,炎性细胞大量浸润,星形细胞增生;PCNSL患者典型的病理特点为瘤细胞围绕血管呈袖套状排列,部分PCNSL患者因使用激素等原因造成病理不典型,与TDL极易混淆,有4例患者进行了2次以上的活体组织检查确诊.结论 （1） PCNSL若在CT上表现为低密度或等密度,需与TDL相鉴别.（2）少数PCNSL患者激素治疗后病理特点与TDL相似,极易误诊,在明确诊断前慎用激素治疗.PCNSL患者激素治疗后疗效不持续.（3）PCNSL的病理表现可能与其病程演进相关,因此对于脑活体组织检查病理不典型者可再次进行活体组织检查.综合临床影像及病理进行诊断具有重要意义,应注重随访.Objective To improve differential diagnosis of tumefactive demyelinating lesions （TDL） and primary central nervous system lymphoma （PCNSL） by analyzing the clinicopathological features of the diseases.Methods The clinical features,neuroimaging findings and pathological characteristics of 4 patients with pathologically proven TDL and 9 patients with pathologically proven PCNSL were retrospectively analyzed.Computer tomography and magnetic resonance imaging were used for neuroimaging studies.The hematoxylin and eosin staining,Luxol Fast Blue staining and immunohistochemistry were used for pathological studies.Results （1） The features of lesions on brain imaging scan：CT in TDL patients showed low density.Enhanced MRI demonstrations were different in different courses：3 cases with ring enhancement,1 case with spotty strengthen;5 PCNSL cases showed hyperdensity in CT,1 case showed isodensity,and 3 cases low-density.MRI showed enhancement of uniform enhancement in PCNSL patients.（2） The features of lesions on pathology：the plaques of lesions in TDL patients were characterized by massive demyelination with relatively axonal preservation associated with prominent astrocytosis and profound infiltrates composed.Typical pathological features in PCNSL cases were that tumor cells around blood vessels showed the cuff-like arrangement.Due to use of hormones and other causes,pathological demonstrations of a part of PCNSL cases were atypical,which were easily confused with TDL.There were 4 cases with more than one biopsy for diagnosis.Conclusions （1） PCNSL with low or equal density in CT needs to be differentiated with TDL.（2） The pathological features of some cases of PCNSL after hormone therapy were similar to TDL.It is better not to use hormone before definite diagnosis.（3） The pathology of PCNSL may be related to the progression of the disease.Some of patients need to be re-biopsied.It is important to combine clinical imaging and pathology for diagnosis of the disease,and attention should be

关 键 词：脱髓鞘疾病 中枢神经系统肿瘤 淋巴瘤 误诊 

分 类 号：R739.4[医药卫生—肿瘤]