儿童室管膜下巨细胞星形细胞瘤

Subependymal giant cell astrocytoma in children:report of 6 cases and review of literature

目的探讨儿童室管膜下巨细胞星形细胞瘤(SEGCA)的临床、影像、病理表现及显微外科手术方法。方法回顾性分析1996年～2004年8年间6例儿童SEGCA的临床资料,6例患儿平均年龄12岁,以癫痫(4/6)和颅内压升高(4/6)为主要症状,MRI表现为侧脑室壁可强化的占位性肿块。结果全部病例采用显微外科手术治疗,无一例死亡,经病理检查证实为SEGCA。术后患儿症状基本消失,随访2～8年,无一例复发。结论儿童SEGCA具有典型的临床表现和MRI特征,作为一种先天性病变,其最佳治疗方法是早期确诊并采取显微外科手术方法切除。

Objective To investigate the clinical, radiological and pathological features of subependymal giant cell astrocytoma （SEGCA） in children,and discuss the outcome of micro-neurosurgical operations. Methods The clinical and pathological data of six patients with intraventricular SEGCAs from 1996 to 2004 were retrospectively analyzed. The six children were males with an average age of 12 years. The patients visited doctor for seizure（4/6）and symptoms of raised intracranial pressure （4/6）. MRI showed obviously enhanced space occupying masses in lateral ventricles. Results All six cases underwent micro-neurosurgical operations. All of them were survived. Pathological diagnosis proved to be SEC, CA. All the patients were followed-up for 2-8 years after operation. Symptoms disappeared with no recurrence in these patients. Conclusions Subependymal giant cell astrocytoma in children has typical clinical and MRI features. Being a congenital disease,the optimal treatment is early diagnosis and total resection by micro-neurosurgical operation.