小儿泌尿系统炎性肌纤维母细胞瘤诊治探讨

Inflammatory myofibroblastic tumors of the urinary tract： a clinical diagnosis and treatment review in pediatric

目的 提高对小儿泌尿系统炎性肌纤维母细胞瘤（inflammatory myofibroblastic tumor,IMT）的诊治水平.方法 回顾性分析2006年1月至2015年7月我院收治的12例小儿泌尿系统IMT的临床资料.男、女各6例.年龄2个月～13岁,平均6.4岁.其中膀胱IMT 8例,肾脏IMT2例,输尿管IMT 1例,前列腺IMT 1例.超声检查显示肿瘤强回声或低回声,边界清楚或不清,血管增粗、增多;增强CT表现为实性边界清楚的肿物,高密度或低密度,肿物周围血管影增粗、增多.MRI增强表现为高密度影.11例行肿瘤切除术,1例前列腺IMT仅行活检.查阅文献总结泌尿系统IMT发生部位及各个部位的诊断治疗方法.结果 肿瘤组织病理HE染色可见大量梭形肌纤维母细胞伴炎性细胞浸润;免疫组化：12例中,8例ALK、SMA阳性,6例CK18阳性,7例Desmin阳性,9例Vimentin阳性;仅2例测Actin,其中1例阳性.Myoglobin、S-100均阴性,Ki-67＜ 20％.10例获随访,随访时间3 ～31个月,平均14.4个月,未见转移及复发,失访2例.结论 小儿泌尿系统IMT罕见,临床及影像学表现缺乏特异性,手术完整切除是主要治疗方法.

Objective To improve the diagnosis and treatment of inflammatory myofibroblastic tumor （IMT） of the urinary tract in pediatric.Methods The retrospective study of 12 IMT was based on information retrieved from Beijing Children＇s Hospital from January 2006 to July 2015.The literatures of urinary IMT were reviewed.There were 12 cases of urinary IMT, with 8 cases in bladder, 2 in kidney, 1 in ureter and 1 in prostate.Mean age at surgery was 6.4 years old （range 2months-13 years）, 6 cases males and 6 females.Tumor resection were performed in 11 patients, biopsy was performed only in 1 patient.Results HE staining revealed diffuse appearing spindle myofibroblastic cells admixed with inflammatory cells.Immunohistochemistry showed positive ration for following markers as ALK （8/12）, CK18 （6/12）, Desmin （7/12）, SMA （8/12）, Actin （1/2）, Vimentin （9/12）.Negative staining were seen for Myoglobin, S-100 and Ki-67 〈 20%.Patients were followed up in 10 cases, lost to follow-up in 2;the mean follow-up time was 14.4 months （range 3-31 months）.All patients recovered well without relapse or metastasis.Condusions Inflammatory myofibroblastic tumors of the urinary tract in pediatric were rare, without specific characteristic in clinical features and imaging.The main treatment of IMT is complete surgical excision.