18例眼部神经纤维瘤的临床病理分析

Clinical and Pathological Analysis of 18 Cases of Ocular Neurofibroma

目的:探讨眼部神经纤维瘤的临床和病理特征,提高临床诊断水平。方法:回顾性分析我院1980年1月至2002年12月手术切除并经病理证实为眼部神经纤维瘤的18例患者的病例资料。结果:单发于眼部者12例12只眼,合并神经纤维瘤病者6例7只眼。最好发的部位是眼睑皮下及眼眶,临床表现为受累部位的占位性病变及累及相关组织的表现。局限型6例,弥漫型3例,丛状型9例。6例丛状型合并神经纤维瘤病,其中4例有全身牛奶咖啡斑。所有病例均行手术治疗,术后7例治愈,其余未痊愈或复发,4例患者行两次以上手术。所有病例均经组织病理学检查确诊。结论:眼部神经纤维瘤伴发神经纤维瘤病者临床易于诊断,单发于眼部的神经纤维瘤主要依靠病理检查确诊。目前治疗还无最佳方法,手术切除应综合考虑,尤其是复发的问题。

Objective：To investigate the clinical and pathological characterastics of ocular neurofibroma and im- prove the diagnostic ability. Methods：Retrospective analysis were performed on 18 cases surffering from ocular neu- rofibroma pathologically diagnosed between Janurary, 1980 to December, 2002 at West China Hospital. Results： There were 12 cases of isolated ocular neurofibroma and 6 cases of neurofibromasis with ophthalmologic manifesta- tion. The eyelid and orbit are the most common site. The patients manifested space occupying lesions and the change of involved tissue. There were 6 cases of localized type,3 cases of diffuse type and 9 cases of plexiform type. 6 cases of plexiform neurofibroma were neurofibromasis,in whom 4 patients had cafe-au-lait maules. All patients were per- formed surgery. Seven patients obtained a cure,other patients did not cure and 4 patients had more than twice sur- gery. Histopathological examination gave the definite diagnoses. Conclusion： It is easy to diagnose ocular neurofibro- ma accompanied with neurofibromasis. To diagnose isolated ocular neurofibroma rely on pathologic examination. There have not good methods to deal with this disease presently. Surgery can remove the affected tissue and make sure the diagnoses, but recurrence must be considered.