主管单位:中华人民共和国
国家卫生健康委员会
主办单位:中国医师协会
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英文作者:Miao Weiwei Wu Xue Ma Liang Jiang Lixue
英文单位:Department of Respiratory Medicine the First Hospital of Harbin Harbin 150010 China
英文关键词:Trachea;Neurofibroma;Tracheoscopy;Interventionaltreatment
目的 总结气管支气管神经纤维瘤患者的临床特点及诊治经过,提高临床医师对该病的认识,减少误诊漏诊。方法 分析哈尔滨市第一医院呼吸内科收治的2例气管支气管神经纤维瘤病患者的一般情况、临床表现、组织病理学、镜下及影像学表现、治疗及预后,并进行相关文献复习。以“气管”和“神经纤维瘤”为检索词检索中国期刊全文数据库、万方数据库及维普数据库,以“Trachea” OR “Neurofibroma”为检索词检索Pubmed数据库,检索时间为1999年1月1日至2017年9月1日。结果 2例患者均为男性,其中1例患者肺部CT可见右主支气管内圆形软组织密度影,支气管镜下可见肿物由右主支气管向上生长,越过隆突阻塞左主支气管管腔约60%;另1例患者肺部CT见气管中上段右侧壁圆形软组织密度影,支气管镜下见气管中上段右侧壁宽基底肿物。2例均经支气管镜综合介入技术切除肿物,病理回报均为神经纤维瘤。目前随访2例患者状态良好,生活工作正常。经上述数据库检索,共检索到14篇文献,其中国外5篇,报道6例患者,大部分为个案报道,国内9篇,均为个案报道。结论气管支气管神经纤维瘤是临床罕见的气管肿瘤,临床表现缺乏特异性,容易漏诊及误诊,诊断依靠组织病理学。放疗及药物治疗均无明显疗效,外科手术是其有效的治疗方法 ,但传统的手术方式创伤较大,随着肺脏病学介入技术的发展,可以采用经支气管镜介入治疗,对气道内的肿瘤具有良好效果。
Objective To summarize the clinical features, diagnosis and treatment of tracheobronchial neurofibroma. To improve clinicians′ understanding of this disease and reduce misdiagnosis and missed diagnosis.Methods The general condition,the clinical manifestations, histopathology, microscopic and imagin findings, treatment and prognosis of 2 patients with tracheobronchial neurofibroma admitted in the First Hospital of Harbin were analyzed. Articles related to "Trachea" and "neurofibroma" were searched in the Chinese Journal Full-text Database, Wanfang Database, Weipu Database, Pubmed Database, and the search time was from January 1st, 1999 to September 1st, 2017. Results Two patients were male, including one patient whose pulmonary CT showed a round soft tissue density shadow on the right main bronchus and bronchoscopy showed that the tumor was around the carina and blocked 60% of the left main bronchial lumen. Another patient showed round soft tissue density shadow in the upper middle section of the right side wall of trachea; bronchoscope showed broad basal mass in the upper middle section of the right side wall of trachea. The pathological findings were neurofibroma of trachea. Two patients had bronchoscopic interventional therapy. Pathological results were all tracheal neurofibroma. Follow-up to date, the 2 patients were in good condition with normal life. Fourteen articles were reported overseas and most of them were case reports; 9 were reported in China and they were all case reports. Conclusions Tracheal neurofibroma is a rare benign tracheal tumor. The clinical manifestations are lack of specificity. The diagnosis relies on histopathology. Radiotherapy and drug therapy show no curative effect. Surgical operation is an effective treatment but the traditional operation is more traumatic. With the development of pulmonology interventional techniques, we can use the bronchoscopic intervention therapy.
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