大脑胶质瘤病临床分析(附17例报告)

Clinical Analysis of Gliomatosis Cerebri

目的:探讨大脑胶质瘤病(gliomatosis cerebri,GC)的临床特征、诊断和治疗方法,提高该病的诊断和治疗水平。方法:对北京市海军总医院1989年1月至2007年10月和吉林大学中日联谊医院2005年1月至2007年12月收治的17例GC患者进行回顾性分析,结合国内外文献分析讨论。结果:17例GC患者临床表现复杂多样,主要表现为T1精WI神呈障低碍信和号性或格等改信变号、改头变痛,、T癫2W痫I呈发广作泛和高偏信瘫号。,影信像号学较检均查匀示,无病或变轻范微围强广化泛。,2C例T呈行稍MR低S密检度查或均低显等示混胆杂碱(密C度ho),上升、胆碱/肌酸(Cho/Cr)和胆碱/N-乙酰天门冬氨酸(Cho/NAA)比值升高。病理学检查示肿瘤细胞广泛浸润大脑半球灰白质,主要沿血管及神经轴突周围呈浸润性生长。17例GC患者中,星形细胞瘤Ⅰ级3例,Ⅱ级8例,Ⅱ～Ⅲ级2例,Ⅲ级4例。总体中位生存期为9个月(21天～24个月)。结论:当患者出现神经系统症状,影像学检查显示弥漫性病灶累及两个以上脑叶,MRI呈长T1、长T2异常信号,增强扫描无强化或轻微强化,MRS示Cho/Cr和Cho/NAA比值升高时,应考虑GC的诊断。最终确诊需行组织病理学检查;立体定向活检术由于其微创、安全、可靠的特点,在大脑胶质瘤病诊断中具有重要的应用价值。GC不适合手术治疗,放疗、化疗及125I籽粒植入间质内放疗对GC有一定的抑制作用,但整体中位生存期短,预后差。

Objective： To investigate the clinical features and methods used to diagnose and treat gliomatosis cerebri （GC）. Methods： Seventeen patients with GC that were diagnosed and treated in Navy General Hospital of PLA from January 1989 to October 2007 and in China-Janpan Union Hospital from January 2005 to December 2007 were retrospectively analyzed. Results： The clinical manifestations of these 17 patients were variable. The main clinical manifestations included headache, mental status changes, seizure and hemiparalysis. Radiological examination revealed diffuse lesions that produced hypodense signal on CT, a hypo- or isoin-tense signal on T1WI, and a scattered diffuse hyperintense signal on T2WI. Contrast enhancement was absent or minimal. MRS revealed increased choline （Cho）/creatine （Cr） and choline （Cho）/N-acetylaspartate （NAA） ratios in tumors compared with normal brain tissues. Histological examination revealed the widespread infiltration of neoplastic glial cells with minimal destruction of pre-existing structures. All cases were astrocytomas including 3 cases of grade Ⅰ, 8 cases of grade Ⅱ, 2 cases of grade Ⅱ - Ⅲ, and 4 cases of grade Ⅲ. The median survival was 9 months （ranged from 21 days to 24 months）. Conclusion： When patients have symptoms and neuroimaging provides MRI evidence of a diffuse infiltrative process involving more than two cerebral lobes that extends to infratentorial structures, a diagnosis of GC should be considered. Stereotactic biopsy is a powerful and safe method to diagnose GC. Surgery is not a suitable option for GC. Radiotherapy, chemotherapy and ^125I seed brachytherapy seem to be helpful, but the median survival is short and the prognosis is still poor.