摘要
目的总结儿童炎性肌纤维母细胞瘤(IMT)的临床发病特征及治疗经验。方法回顾性分析郑州大学附属儿童医院2015年1月至2019年1月病理确诊为IMT患儿的临床资料。结果11例患儿中男4例,女7例,男女比例0.57∶1。发病年龄13 d~11岁,中位发病年龄40.58个月;8例(72.73%)原发于腹腔,3例(27.27%)原发于左肺。肿瘤最大直径为2.5~25.0 cm,中位直径为(7.02±1.43)cm。实验室检查:贫血10例(90.9%),神经元烯醇化酶(NSE)升高5例(45.45%),甲胎蛋白(AFP)升高1例(9.09%),乳酸脱氢酶增高3例(27.27%)。免疫组织化学:间变性淋巴瘤激酶(ALK)阳性6例(54.54%),平滑肌肌动蛋白(SMA)阳性10例(90.90%),S-100蛋白阳性3例(27.27%),结蛋白阳性6例(54.54%),波形蛋白阳性10例(90.90%)。11例患儿均经手术切除局部肿块,8例(72.73%)原发于腹部者仅接受手术切除,4例无病生存,4例带瘤生存,3例(27.27%)原发于肺部者,1例无病生存,2例死亡。结论IMT多原发于腹腔,临床多以局部肿块压迫或浸润临近器官表现为主,相关实验室及影像学检查缺乏特异性。手术完整切除是其主要治疗手段,但局部复发率高,化疗及放疗效果差。
Objective To summarize the clinical characteristics and treatment experience of inflammatory myofibroblastic tumor(IMT)in children.Methods The clinical data of children diagnosed with IMT by pathology in Children’s Hospital Affiliated to Zhengzhou University from January 2015 to January 2019 were retrospectively analyzed.Results Among the 11 cases,there were 4 boys and 7 girls,with a male to female ratio of 0.57 to 1.The onset age ranged from 13 days to 11 years,and the median onset age was 40.58 months.There were 8 cases(72.73%)of primary peritoneal cavity,3 cases(27.27%)of primary in the left lung.The maximum diameter of the tumor was 2.5-25.0 cm,and the median diameter was(7.02±1.43)cm.Laboratory examination showed that anemia accounted for 10 cases(90.9%),neuron-specific enolase(NSE)increased in 5 cases(45.45%),alpha fetoprotein(AFP)increased in 1 case(9.09%),lactate dehydrogenase increased in 3 cases(27.27%).Immunohistochemistry showed that anaplastic lymphoma kinase(ALK)positive in 6 cases(54.54%),smooth muscle actin(SMA)positive in 10 cases(90.90%),S-100 positive in 3 cases(27.27%),desmin positive in 6 cases(54.54%),vimentin positive in 10 cases(90.90%).Local masses were surgically removed in all the 11 children.Eight patients(72.73%)with primary abdominal lesions were excised only,4 patients survived without disease and 4 patients survived with tumor.Among the 3 patients(27.27%)with primary pulmonary disease,1 survived without disease and 2 died.Conclusion IMT mainly occurs in the abdominal cavity.The clinical manifestations of IMT are mainly local mass compression or infiltration of adjacent organs.The laboratory and imaging examinations are lack of specificity.Complete resection is the main treatment,but the local recurrence rate is high and the effect of chemotherapy and radiotherapy is poor.
作者
黄闪
孙红启
刘江华
王利娟
刘炜
HUANG Shan;SUN Hongqi;LIU Jianghua;WANG Lijuan;LIU Wei(Department of Hematology and Oncology,Children’s Hospital Affiliated to Zhengzhou University,Zhengzhou 450018,China;Zhengzhou Key Laboratory of Children’s Infection and Immunity,Children’s Hospital Affiliated to Zhengzhou University,Zhengzhou 450018,China)
出处
《河南医学研究》
CAS
2021年第9期1563-1566,共4页
Henan Medical Research
基金
河南省医学科技攻关计划联合共建项目(LHGJ20200670,LHGJ20190918)。
关键词
炎性肌纤维母细胞瘤
间变型淋巴瘤激酶
治疗
预后
儿童
inflammatory myofibroblastic tumor
anaplastic lymphoma kinase
treatment
prognosis
children
