新生儿和小婴儿肝脏婴儿型血管内皮瘤的诊断治疗探讨

Hepatic infantile hemangioendothelioma in newborns and young infants

目的 探讨新生儿和小婴儿的肝脏婴儿型血管内皮瘤的诊断治疗特点。方法 回顾分析 6例肝脏婴儿型血管内皮瘤并结合文献分析其临床表现、影像学检查、病理特点、治疗方法和疗效。结果 6例中产前B超检查发现肝脏肿块 3例 ,因肝大腹部包块就诊 2例、黄疸 1例。无充血性心衰和Kasabach Merrit综合征等并发症发生。影像学检查 :B超均示肝脏低回声实性肿瘤影 ,血流丰富。CT平扫均示肝脏低密度肿块 ,可有散在或聚集颗粒状钙化 ;增强扫描边缘明显强化 ,逐渐向中心扩张。肿块大小不等。 6例均为单发病变 :肝右叶 4例、左叶 2例 ;肝内 2例、边缘 4例。血甲胎蛋白 (AFP)除 1例未查 ,5例均高于 12 10ng/mL。手术切除 5例 ,1例保守观察。病理特点 :6例均为Ⅰ型血管内皮瘤 :光镜下见由大小不等血管构成 ,管腔内壁可见肿胀增生的内皮细胞 ,核分裂相少见 ;免疫组化凝血Ⅷ因子 (+) ,CD34(+)。随诊 2个月至 2年 :手术患儿无复发 ,3例满 1岁者AFP降至正常 ;1例保守观察者随访至生后 7个月 ,肿瘤略缩小。 6例患儿均生长发育良好。结论 肝脏婴儿型血管内皮瘤发病早 ,多在胎儿晚期、新生儿期及 6个月内发现 ,为良性病变 ,须与肝母细胞瘤和神经母细胞瘤肝转移相鉴别 ;单发病灶无并发症者手术切除效果好 。

Objective To investigate the suitable management of hepatic infantile hemangioendothelioma in newborns and infants.Methods Clinical,pathological and imaging features and the method for therapy were retrospected in 6 children with hepatic infantile hemangioendothelioma.Results Hepatic mass was found preneonately in 3,while hepatomegaly in 2 and jaundice in 1 out of 6 children.No congestive heart failure and Kasabach Merritt syndrome were observed.CT scan showed a typical low attenuation solid lesion with peripheral enhancement image.In 5 children,AFP was more than 1210ng/mL.Tumor resections were performed in 5 and clinical follow up by sonography in 1 patient.Infantile hemangloendothelioma(Pattern Ⅰ)with CD34(+)and Ⅷ factor(+)was found by pathological examination.No recurrence was observed after the operation during the 2 months to 2 years follow up.Conclusion Hepatic infantile hemangioendothelioma is a benign tumor that presents mostly before the age of 6 months.It is necessary to differentiated it with neuroblastoma Stage IVs.Better outcome might be achieved in patients with solitary lesion undergoing surgical resection and in asymptomatic patients with spontaneous tumor regression.