Tuberous sclerosis complex combined with subependymal giant cell astrocytoma: report of 5 cases
10.3760/cma.j.issn.1671-8925.2008.08.012
- VernacularTitle:结节性硬化合并室管膜下巨细胞星形细胞瘤(附五例报告)
- Author:
Yan ZHU
1
;
Rui-Yun LIU
;
Ke-Ming ZHAO
Author Information
1. 环湖医院
- Keywords:
Subependymal giant cell astrocytoma;
Tuberous sclerosis complex;
Microsurgery
- From:
Chinese Journal of Neuromedicine
2008;7(8):802-805
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the subependymal giant cell astrocytoma (SEGA) in detail, on its clinical manifestations, imaging characteristics, pathological subtypes, surgical treatment and prognosis. Methods Five patients with SEGA proved by surgery and pathology were analyzed retrospectively on their clinical manifestations, treatment and prognosis. Results Tuberous sclerosis complex (TSC) combined with SEGA, an autosomal dominant inherited disease, mostly occurred in children. The typical clinical feature was named as Vogt tri-symptoms-sebaceous adenoma, hypophrenia and epilepsy. Because most of tumors were found at the foramcn of Monro, obstructive hydrocephaluseasily occurred due to the obstruction of foramen of Monro and the third ventricle. Five tumors were totally removed by microsurgery without any postoperative complications. No patients received chemotherapy and radiotherapy. Follow-up between 1 and 8 years indicated no recurrence, and all clinical symptoms of these 5 patients were disappeared after operation. Conclusions SEGA belongs to the low-grade intracranial tumors, which has a more satisfied outcome with a low recurrence rate. The major therapy of SEGA is total resection of the tumor by microsurgery, and it has no need to give chemoand radiotherapy after operation.
