Hepatic epithelioid hemangioendothelioma: report of five cases

Results of computed tomography (CT), ultrasonography (US), angiography, and radionuclide imaging were analyzed in five cases of histologically proved hepatic epithelioid hemangioendothelioma (EHE), a rare vascular tumor of adults that has a variable but often long clinical course. All patients received palliative treatment. CT and US were performed repeatedly over a period of up to 53 months. Initial radiologic examination showed two types of hepatic lesions: multiple nodules in both lobes (nodular lesions, n = 2) and large masses with or without calcifications that generally spread along the hepatic margins (diffuse lesions, n = 3). Nodular lesions may be an earlier form of hepatic EHE, as they later gradually changed into the diffuse type. Follow-up CT after treatment showed definite regression of tumors in two cases. In the differential diagnosis, the radiologic findings of diffuse lesions were considered to be specific to hepatic EHE, while those of nodular lesions were nonspecific.     

Hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare, malignant neoplasm of vascular origin arising in soft tissues, lung, and liver. Four cases of hepatic EHE are reported, and 49 previously reported cases are reviewed. The tumor occurs in adults of all ages; 66% of patients are women. The prognosis is variable. Computed tomographic findings suggest the tumor begins as multiple hepatic nodules that grow and coalesce, forming large confluent masses preferentially involving the liver periphery. Extensive hepatic involvement is associated with enlargement of uninvolved portions of the liver and splenomegaly. Awareness of the radiologic features is helpful because clinical findings are nonspecific, and biopsy specimens may be misinterpreted as carcinoma or venoocclusive disease.     

肝脏上皮样血管内皮瘤误诊为转移瘤1例

患者女,50岁.因右上腹不适、隐痛3月,来我院就诊,由于自身原因未行血常规、肿瘤标志物等血液检查,直接要求行CT增强检查,平扫时肝左右叶见多发欠规则形低密度影,边界不清,密度不均,CT值约23 ～ 30 HU,增强扫描后部分病变边缘呈轻度强化,似呈“牛眼征”,肝右叶部分病变呈周边明显异常强化,平衡期呈等或稍高密度改变....     

肺上皮样血管内皮瘤1例并文献复习

患者 男,60岁, 2年前无明显诱因出现左下肋疼痛,呈阵发性胀痛,有麻木感,近5个月疼痛加剧入院.胸部CT:双肺多发沿支气管血管束分布的小结节灶及多发结节状钙化影, 部分小结节内伴点状钙化.左肺下叶后基底段见形态不规则的软组织密度影,大小约2 cm×2.3 cm×3.5 cm,密度不均匀,边缘见多发斑点状钙化,增强扫描病变轻~中度不均匀强化,邻近胸膜肥厚及胸腔少量积液(图1~3).     

肺并发肝上皮样血管内皮瘤影像表现(附3例报告并文献复习)

目的:探讨肺并肝上皮样血管内皮细胞瘤的影像学表现,提高对其认识。方法:回顾性分析3例肺并发肝上皮样血管样内皮细胞瘤,结合文献对其临床表现、影像学诊断、及预后进行分析。结果:该病病因不清,临床可无症状或症状较少且轻微,可表现为咳嗽、咯痰,影像学CT检查主要表现为两肺多发性小结节影,肝内病灶多可见钙化,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。患者预后介于良、恶性肿瘤之间。结论:肺并肝上皮样血管内皮瘤罕见,极易误诊,应予重视。     

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases

Objective

To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma.

Materials and methods

Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the Royal National Orthopaedic Hospital. The imaging and histopathology were reviewed in all cases. Magnetic resonance imaging, which was available from all cases, was assessed for the following features: the number of lesions, location in soft tissue (superficial and or deep/subfascial) and bone, and the signal characteristics and morphology of individual lesions. Immunohistochemistry was performed in all cases to characterize the lesions.

Results

Four of the five patients had multiple lesions involving a single limb. Bone was involved in 3 of the 5 individuals. All tumors diffusely expressed ERG and cytokeratins AE1/3, but not MNF116. CD31 was weakly positive in 4 cases. INI-1 expression was retained in all cases. Imaging features included ill-defined, infiltrative lesions in subcutaneous fat with extension to the adjacent skin, poor- to well-defined intramuscular nodules and predominantly intracortical focal bone lesions with rare medullary involvement.

Conclusion

Pseudomyogenic hemangioendothelioma represents a distinct recently characterized tumor type presenting in young adults, with a tendency towards multicentric bone and soft tissue involvement.     

Spinal epithelioid hemangioendothelioma with epithelioid angiosarcomatous areas

Epithelioid hemangioendothelioma is a distinctive vascular tumor rarely involving bones. We report a case of epithelioid hemangioendothelioma in the cervical spine with angiosarcomatous areas. A 50-year-old female presented with dizziness while walking. A mixed sclerotic and osteolytic or destructive lesion was radiographically disclosed in her upper cervical vertebrae. Histologically, a laminectomy specimen exhibited areas of ordinary epithelioid hemangioendothelioma together with foci of more atypical epithelioid cell proliferation, closely resembling epithelioid angiosarcoma. This phenomenon has been exceptionally described in cases with skeletal lesions, which are typically of low-grade malignancy.This case was presented at the Closed Meeting of the International Skeletal Society, St. Julians, Malta, October 3–5, 2004     

Imaging findings in epithelioid hemangioendothelioma

Purpose-objectiveEpithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE.MethodsRetrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE.ResultsWe evaluated 29 patients (17 F) with median age of 16 years (range 2–76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a “lollipop” sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton.ConclusionEHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and “lollipop” sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.     

FDG PET in epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma (EH) is an uncommon tumor of endothelial origin. It can develop in any tissue and can be multicentric or metastatic. The usual course is a slow progression. Imaging techniques are generally useful in determining the extent of the disease. A case of EH involving bone marrow and mediastinum is described. We discuss the use of FDG PET scanning in EH, showing its use in detecting bone marrow involvement and determining the extent of the disease.     

Imaging findings in epithelioid hemangioendothelioma

Purpose-objectiveEpithelioid hemangioendothelioma (EHE) is a rare vascular malignancy with varying biologic behavior. The purpose of this study was to identify imaging findings most characteristic of EHE.MethodsRetrospective review of clinical and imaging records in patients referred to our Vascular Anomalies Center over a 17 year period with biopsy proven EHE.ResultsWe evaluated 29 patients (17 F) with median age of 16 years (range 2–76 y). The most common presenting symptoms were pain (n = 13) and palpable mass (n = 7). 22 (70%) had multifocal disease. Most common sites of involvement were lung (n = 25), liver (n = 16), bone (n = 12), soft tissue (n = 3) and lymph nodes (n = 1). Of patients with single site disease, 3 had lung, 3 liver, and 1 had bone lesions. In 18/25 with lung disease, there were multiple nodules of varying sizes and characteristics. In 14/16 with hepatic disease there were multiple nodules with predominantly peripheral distribution. Subcapsular retraction was seen in 10/16 and a “lollipop” sign (hepatic or portal vein tapering at the edge of a well-defined hypoenhancing lesion) identified in 5/16. Of 12 osseous lesions, 11 were lytic, 8 involved vertebrae and 9 involved the axial skeleton.ConclusionEHE has varied imaging findings. The most common sites are lungs, liver, and bone, with multi-organ involvement seen in most. Lung disease is most commonly characterized by multiple nodules. Hepatic lesions demonstrate the most distinctive findings, with peripheral distribution, lack of early enhancement, subcapsular retraction and “lollipop” sign. Osseous lesions are commonly lytic and more prevalent in the axial skeleton.     

肝脏上皮样血管内皮瘤的影像学特征

目的：提高对肝脏上皮样血管内皮瘤（EHE）的认识和影像诊断水平。方法：搜集经手术病理证实的5例肝脏上皮样血管内皮瘤患者的CT、MRI和病理学资料,分析和总结其影像学征象。结果：CT平扫示肝脏多个类圆形低密度灶,2例病灶边缘可见条状钙化灶。增强后动脉期主要表现为肿瘤周边轻度强化,延迟后肿瘤实质部分无明显对比剂进入,而中央更低密度区无强化。MRI显示肝内多个类圆形病灶T1WI呈低信号,T2WI呈高信号,病灶边缘有低信号晕环。结论：EHE具有一些特征性的影像学特点,CT和MRI检查能为临床诊断及鉴别诊断提供有价值的信息。     

Scintigraphic features of malignant epithelioid hemangioendothelioma

The scintigraphic features of malignant epithelioid hemangioendothelioma of the liver have not been previously documented. In particular, there are no reports on the nature of gallium uptake in these rare tumors. The case reported is a young woman with primary hepatic epithelioid hemangioendothelioma. Metastatic disease in the skull was detected on a Tc-99m MDP bone scan. A Ga-67 study demonstrated that this tumor was not gallium avid.     

输尿管原发上皮样血管内皮瘤1例

患者 男,30岁,因右肾、右侧输尿管积水3月余以“右肾积水查因”为初步诊断收住泌尿外科.3月前行彩超示右下腹部实性占位,右肾积水并右侧输尿管中上段扩张,未予治疗.既往患痔疮,缺铁性贫血,家族中无类似疾病.入院后先后行相关检查,生化指标:RBC 3.02×109/L;Hb 85.0 g/L;肿瘤标记物:CEA 364.1 U/mL;膀胱镜:右侧输尿管口外上方可见一1.0 cm×1.5 cm左右乳头状瘤体,蒂宽,膀胱右侧壁似有膀胱外肿物受压;肾动态显像:20 min仅见右肾周边少量肾实质显影,其余部位均为放射性空白区,右肾GFR=23.1 mL/min.     

Thoracic epithelioid hemangioendothelioma: imaging and pathologic features

The purpose of this article is to review the imaging features of thoracic epithelioid hemangioendotheliomas with pathologic correlation. The thoracic manifestation of epithelioid hemangioendotheliomas is one of three different CT patterns: multiple pulmonary nodules, multiple pulmonary reticulonodular opacities, or diffuse infiltrative pleural thickening. Without any evidence of histological malignancy, malignant features (multiplicity of pulmonary nodules, hepatic or bone involvement, lymphangitic tumor spread, and infiltrative pleural masses) are characteristic of these infrequent tumors.     

骨上皮样血管内皮瘤的CT、MRI及18 F-FDG PET/CT表现(附2例报道)

目的探讨骨上皮样血管内皮瘤(EHE)的CT、MRI及18 F-FDG PET/CT表现,以提高对该病的影像学认识。方法回顾性分析2例经病理证实的骨上皮样血管内皮瘤患者的CT、MRI及18 F-FDG PET/CT检查图像资料,总结其影像特点。结果2例骨EHE中,1例为全身多发骨骼病变,1例仅为胸11椎体病变。CT表现病灶为边缘清晰,轻度硬化并呈分叶状改变的膨胀性溶骨性骨质破坏,病灶内见散在斑点状高密度影,周边软组织及椎间盘未见明显受累。MRI病灶呈稍长T 1、长T 2信号,其内可见散在斑点状低信号影,增强扫描病灶呈明显不均匀强化。病灶外周可见增强无强化的低信号环。18 F-FDG PET/CT主要表现为病灶部位的溶骨性骨质破坏伴SUV摄取值的升高。结论CT、MRI及18 F-FDG PET/CT检查能帮助评估骨上皮样血管内皮瘤的病灶性质及范围,协助下一步诊疗方案的确定。     

The computed tomographic findings of pulmonary epithelioid hemangioendothelioma

Purpose

The aim of this study is to analyse the computed tomographic (CT) findings of pulmonary epithelioid haemangioendothelioma (EHE).

Materials and methods

The CT features and clinical presentations of six patients (five women, one man; mean age, 53 years) with pathology-proven pulmonary EHE were reviewed. Noncontrast CT images were available for three patients and enhanced CT images for three patients. The image characteristics included the number of tumours, tumour location and size, tumour margins, the presence of calcification/necrosis/cavity, the presence of perivascular location, the presence of pleural lesions, tumour homogeneity at contrast-enhanced CT, tumour enhancement relative to the adjacent muscle and the presence of extrapulmonary lesions.

Results

Multiple nodules/masses with irregular margin were shown in all cases, and reticulonodular opacities and ground-glass opacities were found in one case. Overall, the six cases showed 178 nodules/masses, 90 % (160/178) of which were <1 cm in diameter. The average size of the largest nodules/masses in each case was 2.7 cm. The nodules/masses were mostly (93 %, 166/178) located in the subpleural region (<2 cm from the pleura). A total of 48 % (86/178) of nodules/masses showed punctate calcification in four of six cases. All nodules/masses showed perivascular location. Pleural indentation was shown in all cases, as well as pleural-thickening in five cases and pleural effusion in two cases. On contrast-enhanced CT, EHE showed a mildly heterogeneous hyperdense appearance.

Conclusions

With predilection for subpleural and perivascular location, typical pulmonary EHE appears as multiple irregular nodules with punctate calcification and pleural indentation.     

肝上皮样血管内皮瘤PET/CT影像特点(附2例分析)

目的 探讨肝上皮样血管内皮瘤在PET/CT的影像学特点,提高对其的认识.方法 回顾我院2例肝上皮样血管内皮瘤,对其临床特征、PET/CT特点及预后进行分析.结果 2例PET/CT主要表现为肝内结节呈"葫芦状"或"牛眼征",周围可见晕征及包膜回缩征结构,肝内病变在18F-FDG PET/CT早期轻中度摄取,延迟期都表现出...