婴儿促纤维增生性星形细胞瘤／神经节细胞胶质瘤的诊治体会  

Experience in the treatment of desmoplastic infantile astrocytoma/ganglioglioma

作　　者：赵凤毛[1] 冀园琦[1] 孙骇浪[1] 徐佳童 邓志娟[1] 孙记航[1] 

机构地区：[1]首都医科大学附属北京儿童医院神经外科,100045

出　　处：《中华神经外科杂志》2017年第7期708-711,共4页Chinese Journal of Neurosurgery

摘　　要：目的探讨婴儿促纤维增生性星形细胞瘤／神经节细胞胶质瘤（DIA／DIG）的临床特点、治疗方法以及预后。方法回顾性分析2007年4月至2015年10月首都医科大学附属北京儿童医院神经外科收治的6例DIA／DIG患儿的临床资料。其中男4例，女2例；年龄为2～19个月。其典型影像学表现为大脑半球浅表部位结节状强化灶，囊变多见。肿瘤体积较大，可累及多个脑叶，其中额、顶叶多见。病理学检查显示肿瘤呈神经胶质细胞与神经上皮双向分化，GFAP（＋），S-100（＋），Vimentin（＋），且Ki-67（＋）比例常较低。6例患儿均行一期肿瘤切除。1例术后行硬膜下积液外引流术和分流手术；2例术后仅行硬膜下积液外引流术。随访时间为1-9年。结果6例DIA／DIG患儿（均为WHOI级）均达到肿瘤全切除。3例未行硬膜下积液外引流或分流手术的患儿预后良好，除一过性肌力下降外，未出现其他神经功能障碍和癫痫。3例行二期硬膜下积液外引流或分流手术的患儿中，1例预后良好，1例存在神经功能障碍，1例因感染而死亡。无一例发生脑脊液播散转移及复发。结论DIA／DIG是一类罕见的颅内肿瘤，发病时多〈2岁。肿瘤多累及额叶和顶叶，通常巨大而表浅。影像学方面有其特征性表现。根据其典型病理学表现可确诊。行肿瘤全切除后预后常较理想。Objective To explore the clinical characteristics, treatment and prognosis of desmoplastic infantile astrocytoma/ganglioglioma （DIA/DIG）. Methods A retrospective analysis was conducted from April 2007 to October 2015 on a total of 6 cases with DIG/D1A who were admitted to Department of Neurosurgery, Beijing Childreng Hospital, Capital Medical University. Among them, 4 cases were male and 2 were female, and they were aged 2 - 19 months. The typical imaging findings included nodular enhancement commonly presenting cystic changes in superficial areas of cerebral hemispheres. The size of tumor was relatively large and could involve multiple lobes, in particular, the frontal and parietal lobes. Pathological examination revealed two-way differentiation of tumor cells to glial cells and neuroepithelium with GFAP （ ＋ ）, S-100 （ ＋ ） and Vimentin （ ＋ ）, and Ki-67 （ ＋ ） proliferation index was relatively low. Tumor resection was performed in all 6 cases. Postoperative subdural effusion and shunting were conducted in 1 case, and 2 cases underwent postoperative subdural effusion. The follow-np time ranged from 1 to 9 years. Results All 6 children with DIA/DIG （grade WHO l） underwent total resection of tumors. Among them, 3 cases without undergoing subdural effusion or shunting surgery achieved good outcomes. Except temporary decrease in muscle strength occurring in 1 case, no other neurological disorders or epileptic seizures were reported. Among the 3 cases undergoing subsequent subdural effusion or shunting surgery, 1 case had good outcome, 1 case developed neurologic dysfunction, and 1 case died of infection. No metastasis spread through cerebrospinal fluid or tumor recurrence was observed. Conclusions DiG/DIA is a rare kind of intracranial tumor with common onset at less than 2 years old. The tumors are usually large and located in the superficial areas of the frontal and parietal lobes. DIG/DIA could have its unique imaging characteristics and the diagnosis could be established based on it

关 键 词：神经胶质瘤 疾病特征 治疗 婴儿促纤维增生性星形细胞瘤/神经节细胞胶质瘤 

分 类 号：R739.4[医药卫生—肿瘤]