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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。
Objective: To investigate the clinical features, diagnosis and treatment of side skull base tumors in children. Methods: A retrospective analysis of 8 cases of children with skull base tumor clinical manifestations, imaging features and treatment. Including the temporal and middle and posterior fossa huge trigeminal nerve sheath tumor in 1 case, temporal and middle and posterior fossa melanoma neuroendodermal tumor in 1 case, infantile temporal bone fibroma in 1 case, 1 cases of chordate chordoma, nasal Pharyngeal embryonic rhabdomyosarcoma in 2 cases, neuroblastoma in 2 cases. One case underwent Fusch C type and labyrinth joint pathways through the inferior temporal fossa, one case by the osteotomy, 4 cases by the Fusch C-type path under the infratemporal fossa, one case underwent subtotal tumor resection via the palatal path, one case Simple chemotherapy without surgery. Among the 7 cases of operation, 4 cases had preoperative and postoperative chemotherapy and 3 cases received postoperative chemotherapy. Results: All the seven children survived except one case of chordoma recurrence 5 months after operation (6 cases and 1 case without surgery), of which 2 cases had nasopharyngeal embryonic rhabdomyosarcoma recurrence , A case of neuroblastoma after chemotherapy in children without surgery, 6 months after the recurrence. In addition to 1 case of preoperative abducens nerve involvement and 3 cases of trigeminal nerve involvement, 2 cases had transient cerebrospinal fluid leakage, 1 case of ipsilateral hearing loss, 1 case of hoarseness (ipsilateral vocal cord abduction), 2 cases of dysphagia, After 3 to 4 months gradually recovered. None of the 7 cases of operation had no infection of wound infection and skin flap necrosis. There were no serious complications such as facial paralysis, meningitis, hemiplegia and death. CONCLUSIONS: The tumors in the side of the skull base are deep and the clinical symptoms are complex and diverse. They are occult and atypical. They are often late diagnosed. Positive CT and MRI examination can help improve the early diagnosis rate. Surgical resection of the tumor is still the first choice, before and after surgery with radiotherapy and chemotherapy. Surgical approach should be based on the location of the lesion and the scope of the temporal and temporal fossa joint approach can maximize the removal of invasion of the region of the tumor, preservation of brain function, reduce complications.