Tuberous sclerosis combined with subependymal giant cell astrocytoma

Abstract

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. Key words: Tuberous sclerosis; Subependymal giant cell astrocytoma; Diagnosis; Surgery