小儿腹部炎性肌纤维母细胞瘤诊治探讨

目的探讨小儿腹部炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的诊断与治疗方法,以提高其诊治水平。方法分析首都医科大学附属北京儿童医院肿瘤外科2006—2018年收治的18例经手术病理确诊为腹部IMT患儿的临床资料,包括临床表现、实验室检查、影像学检查、病理学诊断、外科治疗方法及随访情况等。18例患儿术前均行超声检查、CT或MRI检查。1例行穿刺及术前化疗,1例仅行开腹活检及术后化疗,其余均接受肉眼下肿瘤全切术,其中2例弥漫性腹部病变行多次手术并接受全身规律化疗。结果术后病理提示肿瘤细胞均呈梭形伴有炎症浸润,部分镜下可见玻璃样变或局灶出血。1例随访中失访,1例肿瘤位于腹膜后间隙者于术后3个月死亡,其余16例随访4~29个月,未见术后严重并发症或死亡发生,1例弥漫腹部IMT病灶患儿带瘤存活。结论IMT可来源于腹部各区域并引发不同症状。影像学检查利于定位和诊断,但缺乏特异性。大部分单发病变可以单纯经手术治愈,对患有弥漫性腹部病变的难治性病例的治疗亟待经验积累及进一步研究。

Abdominal inflammatory myofibroblastic tumors in children:a clinical diagnosis and treatment review

Objective To explore the diagnosis and treatment of abdominal inflammatory myofibroblastic tumors in children.Methods Retrospective analysis was performed for clinical data of 18 children of abdominal inflammatory myofibroblastic tumors from January 2006 to January 2018.The relevant clinical data included age at diagnosis,size of lesion,presenting symptoms,history of previous illness,radiographic findings,pathological examination and clinical outcomes.The literatures of pediatric abdominal inflammatory myofibroblastic tumors were reviewed.All 18 cases were examined preoperatively by ultrasonography,computed tomography(CT)and magnetic resonance imaging(MRI).The interventions included preoperative puncture plus chemotherapy(n=1),biopsy plus postoperative chemotherapy(n=1)and total resection(n=16).Two cases of diffuse abdominal lesions received postoperative systemic chemotherapy.Results Postoperative pathology showed that tumor cells were fusiform with inflammatory infiltrate and sometimes focal hemorrhage or vitreous degeneration was evident.One case was lost to follow-up and one case with tumor in retroperitoneal space died at 4 months post-operation.During a follow-up period of 4-29 months,the remainder had no postoperative death or complications.One survived with abdominal IMT lesions.Conclusion IMT may occur in any abdominal region with diverse symptoms.Imaging examinations aid its localization and diagnosis but it lacks specificity.Solitary lesions are cured by simple operation.For refractory cases with diffuse abdominal lesions,accumulation of experience and further studies are needed.