摘要
神经纤维瘤病(neurofibromatosis,NF)是一种罕见的神经外胚层疾病,为神经嵴细胞分化异常所致的常染色体显性遗传病,通常可分为Ⅰ型神经纤维瘤病(neurofibromatosis type1,NF1)和Ⅱ型神经纤维瘤病(neurofibromatosis type2,NF2),以NF1发病率为高,达1/3000~1/2699[1]。NF1临床表现多样,可表现为神经纤维瘤、皮肤损害、视神经胶质瘤等,以神经纤维瘤最为常见.
We described a 56-year-old male who was admitted to the hospital due to no sweat on the right side of face for 4 years,and paraesthesia pharynges for 1 month.The physical examination was carried out.And the horizontal local uplift on right lateral wall of pharynx was found with café-au-lait spot scattered all over the right arm.MRI showed a rounded mass on the right parapharyngeal space with clear boundary.Later the tumor was removed by a transoral route with the assist of endoscope.The patient was eventually diagnosed with neurofibromatosis 1 and did not relapse after follow-up.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
北大核心
2020年第8期758-760,共3页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
基金
浙江省医药卫生科技计划面上项目(No:2020KY274)
宁波市医疗卫生品牌学科建设项目(No:PPXK2018-02)。
关键词
咽旁间隙
神经纤维瘤病
内镜外科手术
parapharyngeal space
neurofibromatosis
endoscopic surgical procedures
