儿童幕上原始神经外胚叶肿瘤15例临床分析  

Clinical analysis of supratentorial primitive neuroectodermal tumors in children: 15 cases report

作　　者：唐健[1] 陈隆益[1] 黄光富[1] 王振宇[1] 何永生[1] 

机构地区：[1]四川省人民医院神经外科,四川成都610072

出　　处：《实用医院临床杂志》2009年第5期116-118,共3页Practical Journal of Clinical Medicine

摘　　要：目的探讨儿童颅内幕上原始神经外胚叶肿瘤的临床表现、影像学特征、病理特点及治疗。方法回顾性分析15例经手术和病理证实的儿童颅内原始神经外胚层肿瘤病例的临床资料。结果本组临床表现以慢性颅内高压、神经功能损害以及癫痫发作为主；CT及MRI显示肿瘤体积大，边界清楚，血供丰富，可呈囊性变及瘤内出血，病灶周围无水肿或水肿较轻；在病理上原始神经外胚层肿瘤由小圆细胞构成，可观察到菊花团形成，免疫组化染色显示肿瘤有多向分化的倾向。本组均进行显微外科手术治疗，全切11例，次全切除4例；术后放射治疗11例。好转出院14例，死亡1例。随访10例，生存6—20月，平均12月。结论需结合临床、影像学和病理特点对幕上原始神经外胚层肿瘤进行诊断，治疗以手术切除为首选，结合全脑全脊髓放疗。Objective To discuss the clinical presentation,imaging features,pathological characteristics and therapeutic regimen of supratentorial primitive neuroectodermal tumors in children. Methods Fifteen children with supratentorial primitive neuroectodermal tumors proved by operation and pathology were analyzed retrospectively. Results The main clinical presentations included chronic intracranial hypertension, nervous function damage and epilepsy. CT and MRI imaging showed large,clear bordor and rich blood supply neoplasm. Most tumors contained cysts and hemorrhage. And there were little or mild peripheral brain edema. Homer-Wright type rosettes and small round cells appeared in conventional microcopy. Immunohistochemieal stain revealed both gial and neuronal markers. All patients underwent microsurgery,with total resection in 11 cases and subtotal resection in 4. Eleven patients received postoperative radiotherapy. They were getting better while leaving the hospital in 14 patients and another was died in hospital. Only 11 cases were followed up for 6 months to 20 months with the mean survival time of 10 months. Conclusions It is necessary to combine the clinical presentation, imaging features and pathological characteristics to make the diagnosis of supratentorial primitive neuroectodermal tumors. The therapeutic regimen includes operative resection as the optimal strategy and whole central nervous system radiotherapy as adjuvant treatment.

关 键 词：原始神经外胚叶肿瘤 幕上 儿童 手术治疗 放射治疗 

分 类 号：R739.41[医药卫生—肿瘤] R651.11[医药卫生—临床医学]