Epithelioid Hemangioma (Angiolymphoid Hyperplasia With Eosinophilia) of the Heart With Peripheral Eosinophilia and Nephrotic Syndrome
Epithelioid Hemangioma (Angiolymphoid Hyperplasia With Eosinophilia) of the Heart With Peripheral Eosinophilia and Nephrotic Syndrome
复制标题
DOI：
10.1177/1066896915604254
复制
发表时间：
2016-02-01
影响因子：
1.2
通讯作者：
Llombart-Bosch, Antonio
中科院分区：
文献类型：
--
作者：
Machado, Isidro;Chong, Agustin;Llombart-Bosch, Antonio
上皮样血管瘤(EH)是一种罕见的良性血管病变，其特征是内皮细胞呈上皮样/组织细胞样外观。心脏受累极为罕见。我们报告一位年轻女性的罕见病例，患有室上性心律失常、肾病综合征(膜性肾小球病变)以及妊娠和正常分娩后外周嗜酸性粒细胞增多，类似木村病。超声心动图显示右室有一个大的肿块。心脏肿瘤被切除，组织病理学显示内皮细胞增殖并伴有大量嗜酸性粒细胞。肿瘤内皮细胞呈嗜酸性、多角形，上皮样/组织细胞样形态。偶见淋巴结节。CD34、CD31、ERG和第VIII因子阳性，CK阴性。被诊断为EH。手术切除后，病人活得好好的。EH和木村病是两个独立的实体，但临床和/或组织学上可以观察到重叠。上皮样/组织细胞样内皮细胞构成了有助于EH最终诊断的标志性特征。
Epithelioid hemangioma (EH) is a rare benign vascular lesion, characterized by endothelial cells with epithelioid/histiocytoid appearance. Heart involvement is extremely rare. We present an unusual case of cardiac EH in a young woman with supraventricular arrhythmia, nephrotic syndrome (membranous glomerulopathy), and peripheral eosinophilia after a pregnancy and normal partum resembling Kimura disease. Echocardiogram showed a large tumor mass in the right cardiac ventricle. The cardiac tumor was removed and the histopathology revealed an endothelial proliferation associated with abundant eosinophils. The neoplastic endothelial cells were eosinophilic and polygonal with epithelioid/histiocytoid morphology. Lymphoid nodules were occasionally seen. The neoplastic cells were positive for CD34, CD31, ERG, and factor VIII and negative for CK. A diagnosis of EH was rendered. The patient was alive and well after surgical resection. EH and Kimura disease represent separate entities, but clinical and/or histological overlapping can be observed. Epithelioid/histiocytoid endothelial cells constitute the hallmark feature that favors a final diagnosis of EH.