Epithelioid hemangioendothelioma of the liver

Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. We present two cases of this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Both our cases are highly illustrative of the two ways in which hepatic EH can present (nodular or diffuse) and of its diagnostic and therapeutic management. Neoplastic cells expressed the factor VIII-related antigen, CD31 or CD34. Treatment was surgical resection in one patient and liver transplant in the other. Although EH of the liver has a better prognosis than other hepatic neoplasms, conservative treatment is not recommended. Our cases highlight the importance of a histological diagnosis to avoid it being mistaken for another entity.     

肝上皮样血管内皮瘤1例报告

正1病例资料患者女性,53岁。因间断右上腹疼痛2个月入院。患者于2个月前无明显诱因出现右上腹疼痛,钝痛,呈间断出现,并伴有腹胀,因不影响正常生活,未系统诊治。半个月前出现食欲减退,厌油腻,恶心,无呕吐,就诊于当地医院,行病毒学检查无阳性提示,腹部CT发现肝脏多发异常密度。为进一步诊     

Epithelioid hemangioendothelioma presenting with severe myelofibrosis and a high serum hyaluronan level

Epithelioid hemangioendothelioma (EHE) is a rare tumor originating from the vascular endothelium; it has an intermediate malignant potential. EHEs affect all age groups and mostly originate from the soft tissues of the extremities, lungs, and liver. Spinal EHEs, especially those occurring in the bone marrow region, are extremely rare. We report a case of EHE with massive involvement of the liver, vertebrae, and cranial bones that caused severe myelofibrosis (MF) in a 67-yr-old-male patient. Hyaluronan deposits were diffusely observed in the tumor tissue biopsies obtained from both the liver and bone marrow. Furthermore, the serum hyaluronan level increased markedly along with rapid progression of the disease. To the best of our knowledge, this is the first report of MF occurring in an EHE; hyaluronan may have played an important role in the pathogenesis of fibrosis in this case.     

Epithelioid hemangioendothelioma of the lung (intravascular bronchioloalveolar tumor) in a young girl.

This report describes a 7 year old girl who presented with scoliosis and a small right hemithorax. Findings on spine roentgenographs were pleural thickening on the right and parenchymal densities. Computed tomography of the chest and abdomen revealed multiple pulmonary nodules and two hypervascular lesions within the liver. The diagnosis established by lung biopsy was intravascular bronchioloalveolar tumor (epithelioid hemangioendothelioma of the lung). This is the youngest reported case of this rare tumor.     

CT and MRI diagnosis of hepatic epithelioid hemangioendothelioma

BACKGROUND:Hepatic epithelioid hemangioendothelioma (EHE)is a rare,low-grade malignant vascular tumor.Although its unusual imaging and pathologic findings are being recognized with increasing frequency,diagnosis is still difficult.This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS:Nine patients with hepatic EHE confirmed pathologi- cally underwent plain and dynamic c...     

肝上皮样血管内皮瘤1例

上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)是一种病因及发病机制不明,累及单个或多个器官的血管源性肿瘤,具有低至中度恶性潜能,呈慢性进行性发展过程.肝脏是EHE受累的常见器官之一.Ishak等[1]在1984年首次报道了肝上皮样血管内皮瘤(hepatic epithelioid hemangioendothelioma,HEHE),此后相关报道逐渐增多.影像学检查是诊断HEHE常用的手段之一,如螺旋X线计算机断层摄影术(CT)、磁共振成像(MRI)、核医学检查等,但存在一定的局限性.我们在此报道1例病史长达17年的HEHE患者的肝脏CT灌注特点及其在HEHE诊断中的价值.     

Primary pleural epithelioid hemangioendothelioma (EHE)--two cases and review of the literature

Introduction: We present two cases with symptoms of progressively worsening cough, dyspnea, decreased exercise tolerance and right‐sided back pain in the first case and upper respiratory symptoms characterized by cough and a low grade fever in the second case. Methods: Report of two cases. Results: The initial chest X‐ray in both the cases showed pleural effusion. Further imaging with computed tomography of the chest confirmed the effusion in both cases. Thoracentesis was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary pleural epithelioid hemangioendothelioma was made. Conclusions: Both the cases had progressive clinical deterioration despite chemotherapy with Taxol and Bevacizumab in one case and carboplatin, etoposide, and bevacizumab, in the second case. Both developed metastatic disease to lungs and died. Please cite this paper as: Lazarus A, Fuhrer G, Malekiani C, McKay S and Thurber J. Primary pleural epithelioid hemangioendothelioma (EHE) – two cases and review of the literature. Clin Respir J 2011; 5: e1–e5.     

肺上皮样血管内皮细胞瘤并双侧胸腔积液1例及文献复习

目的 加强对肺上皮样血管内皮细胞瘤的认识,提高诊断率。方法通过对1例肺上皮样血管内皮瘤患者的诊治和文献复习,详细分析该病的病因、临床表现、诊断、鉴别诊断、治疗及预后等。结果该病病因不清,临床症状较少且轻微,影像学检查主要表现为两肺多发性结节影,可累及胸膜并出现胸腔积液;病理学检查主要特征是单细胞原始管腔结构;免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施,患者预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮瘤并双侧胸腔积液罕见,极易误诊,当肺部疾病治疗效果不佳时应警惕其可能性。     

肝上皮样血管内皮瘤患者超声造影特征分析

目的 分析肝上皮样血管内皮瘤（HEHE）的超声造影（CUES）特征变化,旨在为临床诊断提供帮助。方法 2012年1月~2016年12月我院诊治HEHE患者21例,在接受CUES检查前先行常规CT检查,再接受CUES检查。结果 在21例HEHE患者中,肝左右叶均有病灶者11例,肝左叶者为6例,肝右叶者为4例。病灶直径10~35 mm,平均为（25.3±11.5）mm;常规超声发现87个病灶,而超声造影发现126个病灶,其中稍高回声病灶9个,等回声病灶57个,低回声病灶 60个;1例患者病灶存在钙化,3例患者存在病灶相互融合。较大病灶内缘可见短毛刺样增强和环状增强;在动脉期,69个病灶与肝实质同步增强（同进）,31个病灶早于肝实质增强（快进）,26个病灶晚于肝实质增强（慢进）;在增强达峰时,病灶的增强程度低于肝实质者58个,病灶的增强程度等于肝实质者40个,病灶的增强程度强于肝实质者28个;126个病灶均呈不同程度的增强,其增强起始时间为（17.4±5.2）s,达峰时间为（22.0±7.2）s,等回声时间为（23.9±4.6）s,表现为低回声时间为（42.6±13.7）s。在门脉期和延迟期,病灶减退,表现为低回声。结论 CUES检查能帮助诊断HEHE,给临床提供了一种新手段。     

肝脏上皮样血管内皮瘤1例报告

1病例资料患者女性,35岁,因＂间断性右上腹部隐痛1个月,检查发现肝脏占位性病变14天＂入院。既往：检查发现乙型肝炎病原学阳性30余年,未行抗病毒治疗。7年前因急性阑尾炎行阑尾切除术。否认其他病史。查体：皮肤巩膜无黄染,无肝掌,蜘蛛痣,腹部平坦,未见腹壁静脉曲张,右下腹可见一长约8cm陈旧性斜行手术瘢痕,愈合良好,未见胃肠型及蠕动波。腹部未触及明显包块,无压痛、反跳痛、肌紧张,肝脾肋下未触及,     

Hepatic epithelioid hemangioendothelioma: Dilemma and challenges in the preoperative diagnosis

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic to hepatic failure. In addition, laboratory measurements and imaging features also lack specificity in the diagnosis of HEHE. The aim of the present study is to highlight the dilemma and challenges in the preoperative diagnosis of HEHE, and to enhance awareness of the range of hepatobiliary surgery available in patients with multiple hepatic nodular lesions on imaging. In these patients, HEHE should at least be considered in the differential diagnosis.     

Hepatic epithelioid hemangioendothelioma in a young female

Summary Epithelioid hemangioendothelioma in a young female is reported. Her symptoms were right upper quadrant pain and low-grade fever. Laboratory examinations showed an increased number of white blood cells and an elevated erythrocyte sedimentation rate. Plasma factor VII: C was increased. The hepatic lesions were demonstrated as low-density areas by CT scan, and multiple nodules with central depression on the surface of the liver were identified by peritoneoscopy. The biopsy specimens showed spindle-shaped tumor cells set in the fibrous and myxohyalinous tissue. The tumor cells were positive for factor VIII-related antigen and Weibel-Palade bodies. The patient received transcatheter arterial embolization therapy using Gelfoam and mitomycin C following which the plasma level of factor VIII was normalized and regression of the tumors was observed.     

A case report of right atrial epithelioid hemangioendothelioma with multiple pulmonary metastases

Cardiac epithelioid hemangioendothelioma (EHE) is a very rare tumour of endothelial origin with the lung and liver as the most easily metastatic organs. We describe herein a patient with hemoptysis, severe anaemia, and diffuse pulmonary nodules with halo signs that represented metastasis of cardiac EHE; these radiologic manifestations are relatively uncommon. During the initial workup for the patient’s pulmonary nodules, echocardiography missed the cardiac mass. However, positron emission tomography‐computed tomography revealed increased fluorodeoxyglucose intake in the right atrial wall, and cardiac magnetic resonance imaging (MRI) revealed an irregular nodule with normal T1‐weighted signal intensity and hyperintense T2‐weighted signal intensity. Enhanced abdominal computed tomography (CT) revealed micronodular liver metastases. Video‐assisted thoracic surgery was performed to make a definitive diagnosis. Immunohistochemistry staining proved the diagnosis of EHE with positive results for cluster of differentiation (CD) 34, CD31, erythroblast transformation‐specific‐related gene and Ki‐67. The patient started chemotherapy with docetaxel (75 mg/m²) and gemcitabine (900 mg/m²), but this failed to control his disease and he died from an opportunistic infection related to his immunocompromised status 5 months later. For the work out process of bilateral diffuse pulmonary nodules suspicious for cardiac origin, especially with atrial deviation, echocardiography alone is not sufficient to exclude atrial origin. Cardiac CT or MRI might be a better choice.     

以咳血丝痰及反复气胸为主要表现的肺上皮样血管内皮瘤1例并文献复习

目的 总结肺上皮样血管内皮瘤的特征,以提高临床医师对该病的认识.方法 通过对1例肺上皮样血管内皮瘤病例的临床、影像学及病理分析,并复习肺上皮样血管内皮瘤的相关文献,从而总结其特征.结果 首次报道以双肺反复出现气胸为临床表现及双肺逐步出现多个小囊状病灶为影像学特点的肺上皮样血管内皮瘤.结论 对于咳血丝痰、双肺反复出现气胸、影像学表现为双肺多个小结节、散在渗出病变及双肺逐步出现多个小囊状病灶的患者需考虑肺上皮样血管内皮瘤,最终确诊有赖于组织病理学及免疫组化检查.     

肝上皮样血管内皮瘤1例

1．病例资料：患者男，27岁，教师。10余天前于饮酒后出现上腹部不适，以剑突下和肝区最为明显，无腰背部放射痛，无畏寒发热，无恶心呕吐，在当地医院CT检查提示“肝肚多发占位，性质待查”转入本院进一步检查治疗。查体：体温36．8℃，血压135／80mmHg，皮肤黏膜无黄染，无出血点及蜘蛛痣。心肺正常。腹平软，肝脾肋下未及．肝区叩击痛阴胜。腹部移动性浊音阴性．脯鸣青5次/min。     

Long-term durable response to lenalidomide in a patient with hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.     

Epithelioid hemangioendothelioma of the liver: report of two cases

This report describes two patients with hepatic epithelioid hemangioendothelioma: a 33-year-old woman and a 28-year-old man. The first case presented with a palpable abdominal mass and has survived without treatment for 6 years since diagnosis, with ascites but a good general condition. The other patient presented with abdominal pain and cholestasis. He had a rapid course and death occurred 6 months after the onset of symptoms. Histologically the tumors consisted of a proliferation of neoplastic cells with an angiogenic tendency embedded in a myxohyaline stroma. Positivity for the factor VIII-related antigen, for UEA-I and for vimentin and negativity for keratin of the neoplastic cells in the immunohistochemical investigation permitted identification of their endothelial origin. In both cases, the initial diagnosis was erroneous.     

Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy

Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in Kasabach-Merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.