起源于脑干内神经纤维的神经鞘瘤

Schwannomas originated from the nerve fibers in the brain stem

目的总结起源于脑干内神经纤维的神经鞘瘤的临床特点及治疗经验。方法回顾性分析12例起源于脑干内神经纤维的神经鞘瘤的临床资料。头部CT表现为低或混杂密度病变。MRI表现T1加权像为等或低信号,T2加权像为高、低或混杂信号,静脉注射GD-DTPA后,可见明显强化或肿瘤结节样强化。均行手术治疗,根据肿瘤位置选择手术入路,采用远外侧入路6例,后正中入路5例,额颞入路1例。手术切除尽量在肿瘤与周围脑组织分界的界面内进行;如果肿瘤与周围界面不清,或位于重要功能区如延髓,为保证术后病人生存质量,可残留部分肿瘤囊壁。结果肿瘤全切除5例,近全切除7例。病理诊断均为神经鞘瘤。随访2～137个月,平均82.3个月。恢复日常生活及工作10例,生活恢复自理1例,死亡1例。结论起源于脑干内神经纤维的神经鞘瘤术前难以确诊。首选手术治疗,但不易达到肿瘤全切除。即使残留部分肿瘤,多数病人仍可获得较高的生存质量。

Objective To summarize the clinical features and treatment experiences of schwannomas which were originated from nerve fibers in the brain stem.Methods The clinical data of 12 cases of schwannomas originated from nerve fibers in the brain stem were analyzed retrospectively.CT scan of the head showed a low or miscellaneous density lesion.MRI showed hypointense or isointense on T1-weighted images,and hyperintense,hypointense or miscellaneous signals on T2-weighted images.A marked or tumor nodule-like enhancement appeared after intravenous GD-DTPA.All patients underwent operation.The surgical approaches were selected according to the location of tumor,far lateral approach in 6 cases,posterior midline approach in 5,frontal-temporal approach in 1.Tumor removal was confined in the margin of the tumors.If there was no clear margin between the tumor and the brain tissue or the tumor was located at a critical brain area such as the medulla,total removal was not recommended and partial tumor cyst wall persevered to ensure quality of life after operation.Results Tumors were totally removed in 5 cases,subtotal in 7.All the tumors were diagnosed as schwannoma by pathological analysis.During a median follow-up of 82.3 months,ranged from 2 to 137 months,10 patients resumed a normal daily life activity and returned to normal work,1 resumed his self-care ability and 1 died.Conclusions Schwannomas originated from the never fibers in the brain stem may be misdiagnosed preoperatively.The first line therapy is surgery,while it is hard to achieve total removal.Most of the patients have a favorable outcome even if a partial tumor remains.