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0引言神经纤维瘤病(neurofibromatosis,NF)临床上主要分为两种类型,分别为Von Recklin shausen病(NF-Ⅰ型)和双侧听神经瘤型(NF-Ⅱ型)。Von Recklin shausen病为起源于神经嵴细胞分化异常而导致的多系统损害的一种常染色体显性遗传病,占整个NF的90%,患病率为1/3000~1/3500。肿瘤来源于周围神经施万细胞、神经周细胞和成纤维细胞,发生在全身各个部位,可侵犯眼眶和眼球。眼眶神经纤维瘤又称眼眶丛状神经纤维瘤,发病率较低,现报告1例。1病例报告患者,男,4岁,因“左眼睑及面部肿物3a”就诊。病史:
0 Introduction Neurofibromatosis (NF) clinically divided into two types, namely Von Recklin shausen disease (NF-Ⅰ type) and bilateral acoustic neuroma type (NF-Ⅱ type). Von Recklin shausen’s disease is an autosomal dominant genetic disease that is caused by multiple system damage caused by abnormal differentiation of neural crest cells, accounting for 90% of the total NF, with a prevalence of 1 / 3000-1 / 3500. Tumors from the peripheral nerve Schwann cells, pericytes and fibroblasts, occurs in all parts of the body, can encroach on the eyes and eyes. Orbital neurofibroma, also known as orbital plexiform neurofibroma, the incidence is low, is reported in 1 case. A case report patients, male, 4 years old, due to “left eyelid and facial tumor 3a ” treatment. Medical history