胸部炎性肌纤维母细胞瘤临床特征及诊疗分析

Clinical characteristics,diagnosis and treatment of thoracic inflammatory myofibroblastic tumor

目的探讨胸部炎性肌纤维母细胞瘤的临床特点、诊断及治疗经验。方法回顾性分析2012年4月至2019年4月就诊于江苏省人民医院(南京医科大学第一附属医院)病理确诊的发生于胸部的炎性肌纤维母细胞瘤共10例,收集其临床资料、病理资料,并对治疗及预后等情况进行分析。结果10例患者男6例,女4例,平均发病年龄37.9岁。以体检发现比例最高,咳嗽、胸痛、咯血为常见症状。初诊时病变部位右肺6例,左肺3例,纵隔1例。9例患者行外科手术治疗,1例行全麻下气管镜电圈套切除。所有患者均通过免疫组织化学确诊,其中平滑肌肌动蛋白阳性率70%,免疫组织化学检查示间变性淋巴瘤激酶阳性率70%。平均随访35.9个月,1例患者复发。结论炎性肌纤维母细胞瘤具有潜在恶性或低度恶性,症状及影像学缺乏特异性,一旦病理确诊,根治性手术为首选治疗,对于局限性气道内病灶,亦可考虑呼吸介入治疗。

Objective To explore the clinical characteristics,diagnosis and treatment of thoracic inflammatory myofibroblastic tumor.Methods The clinical data,pathological features,treatment and prognosis from 10 patients with thoracic inflammatory myofibroblastic tumor confirmed by pathology were analysed retrospectively from April 2012 to April 2019 at Jiangsu Province Hospital(The First Affiliated Hospital of Nanjing Medical University).Results Ten participants including six males and four females with a mean age of 37.9 years old.Lesions were detected by physical examination in five patients,cough,chest pain and hemoptysis were the common symptoms.A total of 10 lesions including six in the right lung,three in the left lung and one in the mediastinum.Nine patients were treated with surgery,and one patient received high-frequency electrocautery though rigid bronchoscopy under general anesthesia.All the patients were confirmed by immunohistochemistry,positive rate of smooth muscle actin was 70%,positive rate of anaplastic lymphoma kinase was 70%.The mean follow-up time was 35.9 months,and one patient relapsed the other nine patients were cured.Conclusions Inflammatory myofibroblastic tumor is potentially malignant or low malignant,the clinical manifestations and imaging findings are not specific,once confirmed by pathology,radical surgery is the first choice.For the lesion limited to the airway,interventional therapy could be the choice,but close follow up is needed.