儿童侧颅底肿瘤的诊断和治疗

目的:探讨儿童侧颅底肿瘤的临床特点、诊断及治疗方法.方法:回顾性分析8例儿童侧颅底肿瘤的临床表现、影像学特点及治疗方法.其中颞部并中、后颅窝巨大三叉神经鞘膜瘤1例.颞部并中、后颅窝黑色素神经外胚层肿瘤1例,婴幼儿颞骨纤维瘤病1例,斜坡脊索瘤1例,鼻咽部胚胎型横纹肌肉瘤2例,神经母细胞瘤2例.1例经颞下窝Fisch C型及迷路上联合径路、1例经岩骨径路、4例经颞下窝Fisch C型径路将肿瘤全切除,1例经腭径路行肿瘤次全切,1例单纯行化疗未做手术.7例手术患儿中4例术前、术后化疗,3例术后化疗.结果:除1例脊索瘤患儿术后5个月复发死亡外,其余7例患儿均存活(6例手术、1例未手术),其中2例鼻咽部胚胎型横纹肌肉瘤术后小灶复发,1例神经母细胞瘤化疗后未行手术的患儿,6个月后复发.除术前1例外展神经、3例三叉神经受累及外,术后2例出现短暂脑脊液漏,1例同侧听力丧失.1例声嘶(同侧声带外展麻痹),2例吞咽困难,术后3～4个月渐恢复.7例手术患儿均无伤口感染裂开及皮瓣坏死,未出现面瘫、脑膜炎及偏瘫、死亡等严重并发症.结论:儿童侧颅底肿瘤生长部位深在、临床症状复杂多样、隐匿、不典型,往往确诊时已为晚期,积极行CT和MRI检查有助于提高早期诊断率.手术切除肿瘤仍为首选,手术前后配合放、化疗.手术径路的选择应依据病变的部位和范围,颞骨和颞下窝联合径路可以最大限度切除侵及该区域的肿瘤,保存脑神经功能,减少并发症.

Diagnosis and treatment of lateral skull base tumors in pediatric

Objective:To explore the diagnosis and treatment of lateral skull base tumors in children.Method:The clinical data of 8 patients with lateral skull base tumors were reviewed in a retrospective study.One had schwannoma of the trigeminal nerve,one had malignant melanoma,one had fibroma in temporal bone,one had chordoma,two had rhabdomyosarcoma,two had esthesioneurroblastoma.Of 8 patients,one case was treated with chemotherapy.The other 7 cases of benign or malignant tumors underwent surgery in different approaches.Four patients had pre-and postoperative chemotherapy.Three patients received postoperative chemotherapy.Result:The group patients had been followed up for 1-1.5 years and it was shown that there was no recurrence in 4 cases,while there was recurrence in 4 cases.One patient of chordoma died 5 months after surgery,the other 7 patients were alive at the time of analysis.Cerebrospinal fluid leak occurred in two patients who healed with management.Hearing loss occurred in one patient in the operated ear.One patient developed hoarseness.Two patients developed swallowing obstruction and healed 3-4 months after surgery.Conclusion:Tumors in lateral skull base in children were rare,of which malignant tumors were more common compared to benign lesions.CT and MRI are helpful in evaluating extent of disease and preoperative planning.Surgery is the first choice of the treatment for lateral skull base tumors.Radiation therapy and chemotherapy could be used for malignant tumors preoperatively and postoperatively.Combined approach of temporal and infratemporal fossa is suitable for the surgery of those tumors involved temporal bone and middle or posterior cranial fossa as well as infratemporal fossa.