丘脑神经节胶质细胞瘤6例并文献复习

Gangliogliomas arising from the thalamus: Report of 6 cases and literature review

目的探讨丘脑神经节胶质细胞瘤(ganglioglioma,GG)的临床特点、治疗和预后。方法对6例经手术和病理证实的丘脑神经节胶质细胞瘤患者的临床资料进行回顾性分析并复习文献。结果丘脑神经节胶质细胞瘤主要临床表现为颅内压增高和偏身运动感觉障碍,患者平均发病年龄12.5岁。6例肿瘤镜下全切除2例,近全切除3例,大部切除1例。术后3例出现对侧肢体偏身运动感觉障碍加重。6例患者平均随访29个月,2例恢复正常工作,3例生活可自理,1例间变性神经节胶质细胞瘤患者复发后死亡。结论丘脑神经节胶质细胞瘤由于缺乏特异的临床症状和神经影像学表现,术前诊断困难,明确诊断需要组织病理学检查。手术治疗是主要的治疗方法,间变性神经节胶质细胞瘤预后差,术后应长期严密随访。

Objective Ganglioglioma（GG） is a less common primary neoplastic disease of the central nervous system that is typically located supratentorially.The objective of this study was to discuss clinical characteristics,microsurgical treatment and prognosis of gangliogliomas（GG） arising from the thalamus.Methods The hospital data of 6 patients who had undergone microsurgery and were pathologically diagnosed as thalamic GGs were reviewed.Results The main clinical presentations were intracranial hypertension,hemiparesis and impairment of sensation on the contralateral side of the lesion.The mean age at presentation was 12.5 years.The operations consisted of total resection in two,subtotal resection in 3,and partial resection in 1.There were increased hemiparesis and impairment of sensation for three cases.All patients were followed up for a mean of 29 months,of which 2 lived a normal life,three lived independently,one patient with anaplastic GG died of tumor recurrence.Conclusion Because of lacking typical clinical manifestations and neuroimaging characteristics,preoperative diagnosis of thalamic GGs is difficult.Its precise diagnosis mainly depends on histopathology.Surgical resection is the principal treatment,the prognosis of anaplastic GGs is poor,long term and close follow-up is necessary.