结节性硬化合并室管膜下巨细胞星形细胞瘤(附五例报告)

目的 探讨室管膜下巨细胞星形细胞瘤(SEGA)的临床表现、影像学特点、病理类型、治疗方法 及预后.方法 同顺性分析我院收治的5例经手术及病理证实的SEGA患者的临床特点、治疗方法 及预后情况并结合文献复习.结果 结节性硬化(TSC)伴SEGA足一种少见的常染色体显性遗传性疾病,多见于儿童.此病在临床上可表现为典型的Vogt三联征:皮脂腺瘤、癫痫发作和智力障碍.由于肿瘤多发生于室间孔附近,故极易阻塞室间孔及第三脑室引起梗阻性脑积水.本组5例患者均经显微手术全切肿瘤,无术后并发症,随访1～8年肿瘤无复发,临床症状消失.结论 SEGA属于级别低预后好的颅内肿瘤,显微外科手术全切肿瘤是目前主要的治疗方法 ,全切后无需放疗和化疗,亦很少复发.

Tuberous sclerosis complex combined with subependymal giant cell astrocytoma: report of 5 cases

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.