侵袭颅内嗅神经母细胞瘤

Intracranial invasive olfactory neuroblatoma

目的探讨侵袭颅内嗅神经母细胞瘤临床特点及治疗方法。方法对我院神经外科从2001年7月-2005年8月收治的5例侵袭颅内嗅神经母细胞瘤病人的临床表现、影像学特点、病理学特点,手术治疗进行分析,1例行内镜下经鼻活检,4例手术治疗,分别选择经额入路和经额扩展入路,应用不同方法进行颅底重建。全部病人术后行放疗,1例辅助化疗。术后6-45个月进行随访。结果4例侵袭颅内肿瘤全部切除,术后鼻塞全部改善,3例术前视力下降者术后视力均有一定程度的改善,术后均未发生脑脊液漏,2例术后出现腰骶段椎管内转移,分别为术后6-8个月。其中1例手术,死亡1例。结论侵袭颅内的嗅神经母细胞瘤治疗仍然需要多手段进行干预,对侵袭到颅内肿瘤要争取全切除,术后应辅以放疗。

Objective To investigate the clinical characteristics and treatment of intracranial invasive olfactory neuroblastoma. Methods 5 cases of intracranial invasive olfactory neuroblastoma admitted to our department from July 2001 to Aug 2005 were analyzed. The statistical data include clinical feature, radiological and pathological characteristics and operative treatment. Among these 5 cases, one underwent transnasal biopsy, 4 underwent operation. The operative approaches include transfrontal and extended transfrontal approach and skullbase reconstruction was performed. All the patients received radiotherapy and one patient received chemotherapy. Follow - up was performed 6 - 45 months after the operation. Results 4 cases of intracranial invasive olfactory neuroblastoma were resected totally; nasal obstruction was alleviated in all cases; 3 cases had their visual acuity improved; no cerebrospinal fluid （CSF） leakage occurred. Lumbosacral intraspinal metastasis occurred in 2 cases 6 - 8 months after operation separately, among which one received operation again and the other one died. Conclusions Multiple methods are needed for intracranial invasive olfactory neuroblastoma, the tumors invading into cranium should be resected totally as far as possible and the postoperative radiotherapy is necessary for this entity.