儿童及青少年嗅神经母细胞瘤4例及文献复习

Esthesioneuroblastoma in children

目的:总结儿童及青少年嗅神经母细胞瘤(ENB)的诊断和治疗特点。方法:回顾性分析我院1993～2004年经治的4例儿童及青少年ENB的临床资料,并进行文献复习。结果:儿童及青少年ENB以11～15岁发病率最高,主要症状为鼻塞、眼球突出、鼻出血及头痛等;肿瘤主要侵犯鼻腔、筛窦、眼眶和前颅窝。治疗方式主要为手术加放疗,其次为单纯手术。患儿主要因原发病死亡。结论:儿童及青少年ENB少见,其恶性程度高,宜采取综合治疗,预后不良。

Objective：To summarize the clinical characterization of esthesioneuroblastoma in children and adolescence. Method.. The esthesioneuroblastoma in children treated in our hospital from 1993 to 2004 were retrospectively evaluated,and thorough literature review of patients with esthesioneuroblastoma was performed by searching for the key word＂esthesioneuroblastoma＂ in CHKD and in Pub Med between 1994-2004 ,also looking for its reference. Result：The most frequent age stage was 11 to 15 years. The main symptoms were nasal occlusion,proptosis, epistaxis, headache and the main location of disease were the intranasal, ethmoid sinus, orbital, anterior cranial fossa. Treatment was mainly combined surgery and radiotherapy,then surgery alone. Most patients died of primary tumour. Conclusion： Esthesioneuroblastoma in children is very rare and combined surgery and radiotherapy should be selected.