小儿脂肪母细胞瘤19例诊断及治疗分析

Diagnosis and treatment of 1ipoblastoma in children

目的回顾性分析小儿脂肪母细胞瘤的临床资料,以进一步了解该病的临床特点,指导诊治及评估预后。方法回顾性总结首都儿科研究所外科2007年-2012年间收治的脂肪母细胞瘤19例,分析其临床表现、影像学特点、治疗方法、术后病理特征及预后。结果本组病例中男10例,女9例,年龄3个月～11岁。瘤体部位:位于下肢5例,上肢3例,腋部2例,颈及项背部2例,肠系膜2例,骶尾部2例,胸壁1例,臀部1例,阴囊1例。临床上均表现为无痛性软组织肿块。影像学检查提示病灶内组织密度与脂肪组织相仿。病理诊断均为脂肪母细胞瘤。对所有患儿进行了随访,其中2例复发。结论脂肪母细胞瘤是一种少见的软组织良性肿瘤。好发于体表浅部,有包膜或与正常组织边界清楚,也可呈弥漫性或浸润性生长。治疗方法为手术切除。术后虽有复发,但无恶变及转移。

Objective To investigate the diagnosis and treatment of lipoblastoma in children and analyze the clinical manifestations,imaging and pathologic features.Methods Nineteen cases （10 males,9 females,aged 3 months to 11 years） undergone the operation from 2007 to 2012 were enrolled in this study.The clinical characteristics,imaging findings,surgical management and follow-up results were retrospectively analyzed.Results All cases presented with painless soft tissue masses,located in limbs （n =8）,armpit （n =2）,neck （n =2）,mesentery （n =2）,sacrococcygeal region （n =2）,chest wall （n =1）,buttocks （n =1） and scrotum （n =1）.All the diagnoses were confirmed by pathological examinations.Conclusions Lipoblastoma is a rare benign tumor mostly in the subcutaneous and intramuscular areas.Some lesions are circumscribed,and others are infiltrative or diffuse.Surgical resection is the main effective treatment.There have been no reports of malignant transformation or metastases of lipoblastoma,even though a few cases relapse after the surgery.