儿童主支气管炎性肌纤维母细胞性肿瘤1例并文献复习

Inflammatory Myofibroblastic Tumor of the Main Bronchus in a Child: A Case Report and Literature Review

摘要: 炎性肌纤维母细胞瘤(IMT)是好发于儿童及青少年的罕见间叶性肿瘤，支气管内发病尤为少见，临床表现缺乏特异性，易误诊为感染或哮喘。本文报道1例6岁女性患儿，因反复咳嗽半年、加重伴喘息入院，胸部CT提示右主支气管闭塞、右肺不张；电子支气管镜发现右主支气管腔内肿物，予支气管镜下圈套电凝电切术，术后病理结合免疫组化ALK (+)确诊为炎性肌纤维母细胞瘤。术后短期症状缓解，但3个月后复发并出现咯血，遂转至北京儿童医院行胸腔镜中转开胸右上肺切除术，ALK基因FISH检测阳性。术后规律随访至2025年7月未见复发。结合文献复习，本文总结儿童气道型IMT的临床、影像、病理及诊疗特点，提示对迁延不愈、抗感染无效的单侧肺不张、阻塞性肺炎患儿，应尽早行支气管镜检查；支气管镜介入可作为初始诊疗手段，复发或侵袭性病例需及时外科根治切除，ALK靶向治疗为复发、不可切除病例提供新方向。

Abstract: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor predominantly affecting children and adolescents. Endobronchial involvement is extremely rare, and its clinical manifestations lack specificity, leading to frequent misdiagnosis, such as infection or asthma. This paper reports a 6-year-old female girl admitted with recurrent cough for half a year and aggravated wheezing. Chest CT showed occlusion of the right main bronchus and right lung atelectasis. An endobronchial mass was detected by electronic bronchoscopy, and snare electrocoagulation and resection were performed. The diagnosis of IMT was confirmed by pathology combined with ALK (+) immunohistochemistry. Symptoms were relieved shortly after surgery, but recurrence and hemoptysis occurred 3 months later. The patient was transferred to Beijing Children’s Hospital for thoracoscopic converted to open right upper lobectomy, with positive ALK gene detected by FISH. Regular follow-up until July 2025 showed no recurrence. Based on literature review, this study summarizes the clinical, imaging, pathological, diagnostic and therapeutic characteristics of pediatric airway IMT. It suggests that bronchoscopy should be performed as early as possible for children with unilateral atelectasis/obstructive pneumonia that is persistent and unresponsive to anti-infective therapy. Bronchoscopic intervention can be used as an initial diagnostic and therapeutic method, while radical surgical resection is required for recurrent or invasive cases. ALK-targeted therapy provides a new option for recurrent or unresectable cases.