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首页> 外文期刊>Journal of Taibah University Medical Sciences >Regression of multifocal infantile hepatic hemangioendothliomas after steroid therapy
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Regression of multifocal infantile hepatic hemangioendothliomas after steroid therapy

机译：类固醇治疗后多灶性婴幼儿肝血管内皮瘤的消退

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Hepatic hemangioendotheliomas are rare childhood tumors. Infantile hepatic hemangioendothelioma (IHHE) is the most common vascular tumor of the liver in children accounting for 12% of all childhood hepatic tumors. We report an 8-month-old female infant presented at the age of 4 months with incidental hepatomegaly. On presentation her abdomen was soft and lax. The liver was at 8?cm below the right costal margin, not tender with rounded edge and nodular surface. The spleen was not felt and there were no other masses and no ascites. Her liver functions tests were normal. MRI of the liver showed numerous rounded lesions in all the hepatic segments with characteristic of low signal intensity at T1 and high intensity at T2. Post contrast, the lesions showed a peripheral thick rim of enhancement and in the delayed porto-venous phase showed central filling with contrast. The portal and hepatic veins were patent. Our findings were compatible with the diagnosis of multifocal infantile hemangioendothelioma of the liver. Oral prednisone was started for 2 weeks then was tapered over 4 months. A follow up MRI confirmed the decrease in the number and the size of hepatic lesions. A follow-up liver US 24 months after treatment showed residual solitary small lesion in left hepatic lobe measuring 1.2?×?1.0?cm. The authors report that steroid therapy was effective in the treatment of massive multifocal HHE without a need?for other advanced therapies including liver transplantation.
机译：肝血管内皮瘤是儿童时期罕见的肿瘤。小儿肝血管内皮瘤（IHHE）是儿童中最常见的肝脏血管肿瘤，占所有儿童期肝肿瘤的12％。我们报告了一个8个月大的女婴，其在4个月大时出现肝肿大。介绍时她的腹部柔软而松弛。肝位于右肋缘以下8？cm，不嫩，边缘圆润，结节状。没有感觉到脾脏，没有其他肿块和腹水。她的肝功能检查正常。肝脏的MRI显示所有肝节段都有许多圆形病变，其特征在于T1处的信号强度低，T2处的信号强度高。对比后，病变表现出周围增强的厚边缘，并且在延迟的静脉-静脉相中表现出具有对比的中央填充。门静脉和肝静脉为专利。我们的发现与婴儿多灶性肝血管内皮瘤的诊断是一致的。口服泼尼松开始2周，然后在4个月内逐渐减量。后续的MRI证实了肝脏病变的数量和大小的减少。治疗后24个月的US随访显示，左肝叶中残留的孤立性小病变为1.2？×？1.0？cm。作者报告说，类固醇疗法可有效治疗大规模多灶性HHE，而无需其他先进疗法，包括肝移植。

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