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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 ,无症状者可以保守观
Objective To investigate the diagnosis and treatment of liver infantile hemangioendothelioma in neonates and infants. Methods Retrospective analysis of 6 cases of liver infantile hemangioendothelioma combined with literature analysis of its clinical manifestations, imaging studies, pathological features, treatment and efficacy. Results 6 cases of prenatal B-ultrasound found in 3 cases of liver mass, liver mass in 2 cases, jaundice in 1 case. No congestive heart failure and Kasabach Merrit syndrome and other complications. Imaging examination: B ultrasound showed liver hypoechoic solid tumor shadow, rich blood flow. CT scan showed low-density lumps of the liver, there may be scattered or aggregated granular calcification; enhanced edge significantly enhanced scan, and gradually expanded to the center. Mass sizes. 6 cases were single lesions: 4 cases of right lobe and 2 cases of left lobe; 2 cases of liver and 4 cases of margin. Blood α-fetoprotein (AFP) except one case were not investigated, 5 cases were higher than 12 10ng / mL. Surgical resection in 5 cases, 1 case of conservative observation. Pathological features: 6 cases were type Ⅰ hemangioendothelioma: seen by the light microscope, ranging in size from the blood vessels, the inner wall of the lumen can be seen swollen endothelial cells, mitotic phase rare; immunohistochemical coagulation Ⅷ factor (+), CD34 (+ +). The patients were followed up for 2 months to 2 years: no recurrence was found in the operation, and 3 cases of AFP were reduced to normal in 1-year-old children. One case of conservative observer was followed up for 7 months and the tumor was slightly reduced. 6 cases of children all grow well. Conclusion The incidence of infantile hemangioendothelioma in the liver is early and mostly found in the late fetus, neonatal period and within 6 months. It is a benign lesion and must be differentiated from hepatoblastoma and neuroblastoma liver metastases. Surgical resection effect is good, asymptomatic people can conservative view