结节性硬化合并室管膜下巨细胞星形细胞瘤

目的 探讨室管膜下巨细胞星形细胞瘤的临床特点、诊断方法和治疗原则.方法 1996年9月至2006年4月,共17例室管膜下巨细胞星形细胞瘤患者,其中采用经胼胝体入路11例,经额部皮质造瘘入路6例;所有患者均进行随访,随访时间为3-123个月.结果 17例患者共18个肿瘤,全切15例,大部切除2例,1例患者未行手术治疗,2年后死亡,1例患者在手术后3个月死亡.有3例患者在手术后需要分流.其余15例患者目前均存活良好,无手术相关并发症,肿瘤目前无复发.未见到放疗对肿瘤生长有影响.结论 手术治疗是该肿瘤惟一有效的治疗方法,经胼胝体入路和经额人路均可以很好地切除肿瘤.

Tuberous sclerosis combined with subependymal giant cell astrocytoma

Objective To describe the clinical features, diagnosis and treatment of subependymal giant ceil astrocytoma. Method Out of 17 TSC patients admitted to Tiantan Hospital from September 1996 to April 2006, 17 tumors out of 16 patients were removed surgically. 11 tumors were surgically resected through transcallosal approach, 6 through frontal transcortical approach. Follow-up ranged from 3 months to 123 months. Results 1 case died 2 years later without operation. Out of 17 pathologically confirmed SEGAs from 16 patients, 15 tumors were totally removed,2 tumors were subtotally removed. 1 case died 3 months after operation, excellent outcomes occurred in other 15 patients without long-term complications. It was necessary to insert a postoperative shunt in only 3 patients. There were no recurrences of 15 SEGAs that were totally removed after a mean follow-up of 41.2 months. Radiotherapy was proved ineffective. Conclusions Surgical removal is the only effective treatment of SEGA. Transcallosal approach and frontal transcortical approach are both effective.