儿童心脏横纹肌瘤的影像学评价及与结节性硬化症的关系  

Imaging diagnosis of cardiac rhabdomyoma and its relation with tuberous sclerosis complex

作　　者：王瑞珠[1] 唐文伟[1] 刘浩[2] 丁培成 杨明[1] 

机构地区：[1]南京医科大学附属南京儿童医院放射科,江苏南京210008 [2]南京医科大学附属南京儿童医院超声科,江苏南京210008 [3]南京医科大学附属南京儿童医院胸外科,江苏南京210008

出　　处：《实用放射学杂志》2016年第11期1762-1765,共4页Journal of Practical Radiology

摘　　要：目的分析儿童心脏横纹肌瘤（cR）的影像学表现及其与结节性硬化症（TSC）的关系，提高对该病的认识。方法回顾性分析在本院就诊的CR24例（其中13例合并TSC）的临床及影像学资料，总结其影像学特征及与TSC的关系。结果24例心脏CR患儿共发现心脏多发肿瘤49处，以心室壁、室间隔、流出道为好发部位，超声为卵圆形稍强回声结节，与心肌分界欠清楚，CT平扫多发结节影与心肌密度相仿，增强后轻度强化，3例合并流出道梗阻。合并TSC者头颅MRI见室管膜下多发结节，皮层和皮层下多发异常信号。结论CR在儿童心脏肿瘤中最多见，常多发，超声心动图和CT各有优势。CR与TSC关系密切，发现CR需考虑是否合并TSC，反之，发现TSC患儿，亦需检查心脏，判断是否合并CR。Objective To analyze the imaging feature of pediatric cardiac rhabdomyoma （CR） and the relationship between cardiac rhabdomyoma and tuberous sclerosis complex （TSC）. Methods The imaging and clinical findings of 24 cases with CR （13 cases combined with TSC） from our hospital were analyzed retrospectively. Also, we analyzed the relationship between pediatric CR and TSC,and summarized the imaging features of pediatric CR. Results 49 multiple tumors were found in 24 cases with CR, with predi- lection sites of ventrlcular wall, ventricular septum and outflow tract. The tumors were oval slightly hyperechoic nodules, and demarcation was clear with normal myocardium. The density of multiple nodules were similar to that of the myocardium in CT scan, and increased with mild enhancement. Among them,3 cases had outflow obstruction. The brain MRI of patients with CR combined with TSC showed bilateral subependymal nodules, subcortical white matter and cortical tubers. Conclusion The incidence of CR is hightest in pediatric cardiac tumor which is ofen multiple and its imagings are characteristics while echocardiography and CT have their own advantages. CR is closely related with TSC. When there are imaging findings of cardiac rhabdomyoma, the possibility of TSC should be considered,and vice versa,all TSC children should be examined to confirm the existence of CR.

关 键 词：横纹肌瘤 结节性硬化症 超声心动图 计算机体层成像 

分 类 号：R732.1[医药卫生—肿瘤] R445.1[医药卫生—临床医学]