原发性中枢神经系统淋巴瘤的临床特点和影像特征分析

Clinical and imaging features of primary central nervous system lymphoma

  • 摘要:
      背景  过去认为原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)患者以免疫功能缺陷最为常见,然而近年来免疫功能正常的PCNSL患者比例逐渐升高,且呈年轻化趋势。研究免疫功能正常的中枢神经系统淋巴瘤患者的病例特点有助于提高对原发性中枢神经系统淋巴瘤的认识,从而提高早期诊断率。
      目的  研究原发性中枢神经系统淋巴瘤的临床及影像特点,为临床诊断提供参考。
      方法  收集2001年1月- 2019年9月就诊于解放军总医院第一医学中心经病理证实的原发性颅内淋巴瘤患者51例,对其临床特点和影像特征进行回顾性分析。
      结果  共收集51例PCNSL患者,其中女性20例,男性31例,男女比例为1.6∶1,平均发病年龄为(53.5±11.6)岁。35.3%的患者急性起病,以头痛(51.0%)、头晕乏力(33.3%)等非特异性症状为首发表现的比例较高,初次诊断时误诊率达29.4%;随着病情进展,患者运动功能、认知功能及眼部受累比例明显升高。病灶位置以额叶(51.0%)、颞叶(39.2%)多见。70.6%患者的灶周呈中重度水肿,62.5%患者的病灶呈中重度占位效应。多发病灶较常见(72.5%),额叶受累的多发病灶组(62.2%)显著高于单发病灶组(21.4%)。47.8%患者病灶在CT上呈高密度,82.9%患者病灶T1WI呈低信号改变,78% T2WI呈高信号改变,71% DWI呈高信号,52.6%呈均匀强化。
      结论  PCNSL好发于中年男性,以头痛、头晕、乏力等起病多见,随着病情的加重,运动系统、认知功能及眼部受累比例增加。MRI病灶多发较常见,以额叶居多,病变DWI高信号、T1WI低信号以及强化多见,多数伴有不同程度的水肿及占位效应,临床医生应熟练掌握PCNSL的临床和影像特征,以提高早期诊断的准确率。

     

    Abstract:
      Background  In the past, primary central nervous system lymphoma (PCNSL) patients with immunodeficiency have been the most common. However, in recent years, PCNSL cases with normal immune function are increasing, and the age of onset shows a younger trend. The study of the characteristics of patients with normal immune function of central nervous system lymphoma is helpful to improve the understanding of primary central nervous system lymphoma and improve the rate of early diagnosis.
      Objective  To analyze the clinical and imaging features of PCNSL, and provide evidence for clinical diagnosis.
      Methods  A total of 51 cases of primary intracranial lymphoma confirmed by pathology in the First Medical Center of Chinese PLA General Hospital from January 2001 to September 2019 were collected, and their clinical and imaging features were analyzed retrospectively.
      Results  Of the 51 cases, there were 31 males and 20 females, with a male-to-female ratio of 1.6 to 1. The average age of onset was (53.5 ± 11.6) years. About 35.3% of the patients had acute onset, and the proportion of patients with non-specific symptoms such as headache (51.0%), dizziness and fatigue (33.3%) was high, and the initial misdiagnosis rate was 29.4%. With the progress of the disease, the proportion of patients with motor dysfunction, cognitive dysfunction and eye involvement increased significantly. Most of the lesions were in frontal lobe (51%) and temporal lobe (39.2%). Moderate and severe edema showed in 70.6% of patients, and 62.5% of patients had moderate and severe space occupying effect. Multiple lesions were more common (72.5%). The frontal lobe involvement of multiple lesions group (62.2%) was significantly higher than that of single focus group (21.4%). CT imaging showed high density in 47.8% of the patients, T1WI showed low signal in 82.9% of patients, T2WI showed high signal in 78% of the patients, DWI showed high signal in 71% of the patients, and uniform enhancement in 52.6%.
      Conclusion  PCNSL is more common in middle-aged men, with headache, dizziness and fatigue as common onset symptom. With the aggravation of the disease, the proportion of motor system, cognitive function and eye involvement increase significantly. MRI show that multiple lesions are more common, mostly in the frontal lobe, with high signal intensity on DWI, low signal intensity and enhancement on T1WI, most of which are accompanied by edema and space occupying effect in different degrees. The misdiagnosis rate of early diagnosis is high. Clinicians should skillfully master the clinical and imaging features of PCNSL in order to improve the accuracy of early diagnosis.

     

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