Malignant Hepatic Epithelioid Hemangioendothelioma with Rapid Progression and Fatal Outcome
Malignant Hepatic Epithelioid Hemangioendothelioma with Rapid Progression and Fatal Outcome
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DOI：
10.2169/internalmedicine.49.3148
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发表时间：
2010-01-01
影响因子：
1.2
通讯作者：
Hachiya, Tsutomu
中科院分区：
文献类型：
--
作者：
Komatsu, Yoshimichi;Koizumi, Tomonobu;Hachiya, Tsutomu
一位46岁女性因右上腹疼痛及呼吸困难而入院。腹部和胸部计算机断层扫描（CT）显示两个肝叶低密度区域、左胸腔积液和两肺多发结节。实验室数据显示弥散性血管内凝血。尽管进行了包括机械通气在内的强化治疗，患者仍发生了快速进行性呼吸衰竭和肝功能衰竭，并在入院后3周死于呼吸衰竭。肝脏尸检的免疫组织化学分析和左侧胸腔积液的细胞学检查，因子VIII相关抗原和CD 31染色阳性。根据这些观察，诊断为血管内皮瘤（EHE），一种罕见的血管肿瘤。快速的临床过程和致命的结果，如在这种情况下，是罕见的临床表现EHE。
A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.