多发椎管内占位起病的Ⅱ型神经纤维瘤病1例并文献复习

【摘要】目的探讨分析Ⅱ型神经纤维瘤病(NF2)的临床表现，特别是椎管内占位的特点、手术治疗的原则及疗效。方法回顾性分析1例以多发椎管内占位起病的Ⅱ型神经纤维瘤病患者的临床资料，结合相关文献进行分析。结果本例患者行多发椎管内占位切除后恢复良好，术后无明显并发症，术前症状得到缓解，随访未见肿瘤复发。结论Ⅱ型神经纤维瘤病患者的治疗需个体化，对产生明显占位效应的肿瘤可采取手术切除,但往往难以将多发的肿瘤完全切除，随着分子生物学的发展未来有望得到根治。

Abstract: ObjectiveTo investigate and analyze the clinical manifestations of neurofibromatosis Ⅱ(NF2), especially the characteristics of space occupying in spinal canal, the principle and curative effect of surgical treatment. MethodsThe clinical data of a patient with NF2 developed from multiple intraspinal space occupying lesions were analyzed retrospectively. The relevant literature were reviewed. ResultsThe patient recovered well after multiple intraspinal space occupying lesions resection, there were no obvious postoperative complications, the preoperative symptoms were relieved, and there was no tumor recurrence during follow-up. ConclusionsThe treatment of patients with NF2 needs to be individualized. Tumors with obvious space occupying effect can be surgically removed, but it is often difficult to completely remove multiple tumors. With the development of molecular biology, it is expected to be cured in the future.