Kim Geum Ha, Kim Yun Soo, Kim Hyun Ok, Kim Kil Hyun, Hung Young Kul, Jung Dong Hae, Kim Jeong Ho, Kwon Oh Sang, Choi Duck Joo, Kim Ju Hyun
Department of Internal Medicine, Gil Medical Center, Gachon University of Medicine and Science, Incheon, Korea.
Korean J Hepatol. 2009 Dec;15(4):510-6. doi: 10.3350/kjhep.2009.15.4.510.
Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.
原发性肝上皮样血管内皮瘤是一种罕见的起源于内皮的肿瘤。其临床表现无特异性，从完全没有症状到肝功能衰竭和死亡不等。肝上皮样血管内皮瘤的自发破裂是一种极其罕见的表现。我们报告一例65岁男性酒精性肝硬化患者的原发性肝上皮样血管内皮瘤病例。他因上腹部疼痛和腹部超声检查发现多个肝脏肿块而住院。肝脏动态CT成像显示多个外周结节状强化肿块病变，呈延迟向心性强化，以及沿肝包膜的高衰减液体聚集。腹腔穿刺发现腹腔内有血液。根据组织病理学检查显示CD34免疫组化染色阳性，最终诊断为原发性肝上皮样血管内皮瘤伴自发破裂。
