舌下神经鞘瘤的诊断和治疗

Hypoglossal neurilemmoma: current experience in diagnosis and treatment

目的 提高舌下神经鞘瘤的诊治水平。方法 回顾性分析 10例舌下神经鞘瘤患者的临床表现、影像学特征、诊断、治疗和随访情况 ,并对我科早期 5例和近期 5例舌下神经鞘瘤的治疗方案及结果进行比较。结果 10例患者均有患侧舌肌萎缩表现 ,颅底CT薄层扫描可以显示骨性舌下神经管 ,但肿瘤较小时不一定能判定肿瘤的存在 ,MRI是最佳的诊断措施。早期 5例均为哑铃型肿瘤 ,其中 2例采用分期手术分别切除肿瘤的颅内部分和颅外部分 ,3例经远外侧入路切除肿瘤 :1例经髁入路 ,2例经髁上入路 ;肿瘤全切除 1例 ,次全切除 4例 ;术后 1例并发脑脊液漏 ,颅内感染 ;Karnofsky预后评分 :良 4例 ,死亡 1例。近期 5例 ,其中 4例 ( 2例哑铃型 ,1例颅内型和 1例管内型 )经改良远外侧入路切除肿瘤 :经枕髁入路 1例 ,经髁上入路 3例 ;1例颅外型肿瘤 2次经颈入路切除肿瘤 :首次手术大部切除肿瘤 ,肿瘤残余部分行伽马刀治疗无效后在导航及内窥镜辅助下再次手术全切除肿瘤 ;肿瘤全切除 4例 ,次全切除 1例 ;术后无并发症发生 ;失访 1例 ,其余 4例Karnofsky预后评分 :优 3例 ,中 1例。近期 5例疗效优于早期 5例。结论 舌下神经鞘瘤罕见 ,手术难度大。根据肿瘤部位和大小设计手术方式 ,尽可能一期全切肿瘤 ,减少侵袭性操作对提高治疗?

Objective To improve the diagnosis and treatment of hypoglossal neurilemmoma (HGN). Methods The data of 10 patients with HGN were retrospectively reviewed, 5 of 10 patients (early group ) had been reported previously. A comparison of treatment strategy between early and latter group was made. Results Typical hemiatrophy of the tongue presented in all 10 patients, hypoglossal canal could be showed in the bone window of CT , which , however ,might not confirm the existence of tumor if it is very small. MRI was the optical choice for diagnosis. Among the early 5 cases with HGN which were all of dumbbell type , the intracranial part and the extracranial part of the tumor in 2 cases were removed stagedly , 3 cases were operated via the far-lateral approach ,of which 1 was via transcondylar approach and 2 were via trans-supracondylar approach ; the 5 tumors were removed subtotally except 1 totally; CSF leakage and intracranial infection after operation occurred in 1 case ; the rating of Karnofsky Prognosis Scale was good in 4 cases and dead in 1 case. However, among the latter 5 cases, 4 cases including 2 of dumbbell type ,1 of intracranial type and 1 of intracanal type were operated via the modified far-lateral approach, of which 1 was via transcondylar approach and 3 were via trans-supracondylar approach ;and the tumor of extracranial type in the last case was resected twice via transcervical approach , being removed subtotally in the first operation followed by total removal with the aid of neuroavigation and nueroendoscope in the second operation when it failed to react to the treatment of gamma knife ;the 5 tumors were removed totally except 1 subtotally.;all postoperative courses were uneventful.;follow up was performed successfully in 4 cases, the rating of Karnofsky Prognosis Scale was excellent in 3 cases and fair in 1 case. The outcomes of the latter 5 cases resected via modified approach were better than those of the early 5 cases. Conclusion HGN is extremely rare and it is difficult to treat it. To achieve a good operative outcome , it is very important to design the approach individually based on its location and size and operate mininvasively and resect tumor totally as possible as can in the primary stage.