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。     

肺上皮样血管内皮瘤(附1例报告及文献复习)

目的提高对肺上皮样血管内皮瘤的诊断和治疗。方法通过对1例肺上皮样血管内皮瘤患者的诊治,就本病的临床特点、诊断方法以及治疗和预后等情况进行全面的分析和讨论。结果肺上皮样血管内皮瘤是一种罕见的肺部肿瘤,病因不明,以干咳为主,影像学表现主要为双肺弥漫性小结节影,病理学检查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,恶性程度并不低,疗效差。结论肺上皮样血管内皮瘤是一种罕见的肺部恶性肿瘤,临床无特异性,极易误诊,目前尚无确切的治疗方法,预后差,临床医生应该提高对其的认识程度。     

肺上皮样血管内皮细胞瘤1例并文献复习

目的加强对肺上皮样血管内皮细胞瘤的认识,提高诊断率。方法结合广州呼吸疾病研究所2004-05-26收治的1例肺上皮样血管内皮细胞瘤患者的临床资料和文献复习,详细分析该病的病因、临床特点、病理诊断、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,多为咳嗽、咳痰、少量咯血;影像学检查主要表现为两肺多发性结节影;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,预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮细胞瘤罕见,极易误诊,应予重视。     

胸膜上皮样血管内皮瘤的研究进展

上皮样血管内皮瘤是一种罕见的血管肿瘤,来源于胸膜的胸膜上皮样血管内皮瘤(pleural epithclioid hemangioendothelioma,PEH)更为罕见.PEH的病因及发病机制尚不清楚,主要临床表现为胸痛、咳嗽和呼吸困难,影像学表现主要为胸腔积液及胸膜增厚,组织学特征主要为上皮样瘤细胞及血管内皮标志物CD31、CD34阳性,目前尚未建立有效的治疗方案.本文就该病的病因、临床特征、诊疗特征及预后进行综述,以提高人们对PEH的认识,加强对其发病机制与诊治的研究.     

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.     

肺上皮样血管内皮细胞瘤1例并文献复习

目的 加强对肺上皮样血管内皮细胞瘤(PEH)的认识,提高诊断率.方法 结合我院诊治的1例PEH患者的临床资料和文献复习,分析该病的病因、临床特点、病理诊断、鉴别诊断、治疗及预后.结果 PEH病因不清,临床症状较少且轻微,多为咳嗽、咳痰、少量咯血;影像学检查主要表现为两肺多发性结节影,病理学检查主要特征是单细胞原始管腔结构;免疫组织化学检查提示其为内皮细胞源性.目前尚无有效治疗措施,预后介于良、恶性肿瘤之间.结论 PEH是一种低度恶性肿瘤,好发于中年女性,临床表现无特异性,易误诊,需肺活检才能诊断.     

肺母细胞瘤1例并文献复习

目的提高对肺母细胞瘤的认识。方法结合1例肺母细胞瘤患者的临床资料和文献复习,详细分析该病的组织起源、临床病理特点、分类、诊断、鉴别诊断、治疗及预后等。结果该病临床症状少且轻微,影像学提示“良性肿瘤”;病理学检查主要特征是镜下瘤组织由胎儿型腺管样的上皮成分和原始的肉瘤样间质成分混杂存在。免疫组化示瘤组织由上皮和间叶两种成分构成。目前尚无有效治疗措施,预后差。结论肺母细胞瘤罕见,极易误诊,应予重视。     

肺硬化性血管瘤15例临床分析并文献复习

目的提高对肺硬化性血管瘤的认识。方法结合15例肺硬化性血管瘤患者的临床资料和文献复习，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，组织来源不明，临床症状较少且轻微，影像学检查主要表现为肺部孤立、境界清楚的结节影。病理学检查主要特征是：（1）实性细胞团及黏液样基质内散在有白细胞；（2）血管瘤样增生伴有管壁硬化倾向；（3）增生的小血管呈乳头状突向肺泡腔内；（4）存在出血及硬化区。免疫组织化学至今不能确定其组织来源。肺硬化性血管瘤术前难于确定诊断，而临床上与肺恶性肿瘤有类似症状，13例术前诊断为恶性肿瘤，误诊率高。但本病尚属良性肿瘤，宜早期手术。手术预后均良好。结论肺硬化性血管瘤发病率低，极易误诊，应予重视。     

肝脏上皮样血管内皮瘤MRI表现分析

目的探讨肝脏上皮样血管内皮瘤的MRI表现及其病理基础,提高诊断准确率。方法回顾分析2012年3月至2017年5月解放军第三○二医院收治的8例经病理证实的肝脏上皮样血管内皮瘤患者的MRI影像特征和临床资料,由两名高年资主治医师共同分析其MRI表现并结合病理分析。结果 8例肝上皮样血管内皮瘤患者肝内病灶均为多发,3例伴双肺转移,1例同时有双肺和椎体转移。MRI检查肝内病灶T1WI呈低信号,T2WI呈稍高信号,增强扫描呈渐进性强化或呈环状强化,部分可见"包膜回缩征"或"棒棒糖征"。结论肝脏上皮样血管内皮瘤的MRI表现具有一定特点,但确诊仍须活组织检查。     

肺上皮样血管内皮瘤1例并文献复习

上皮样血管内皮瘤是一种比较罕见的肿瘤,为血管源性,恶性程度较低或介于良恶性之间,多发生于软组织,最早报道为发生于肺的上皮样血管内皮瘤,之后有其他部位的血管内皮瘤报道。肺上皮样血管内皮瘤（pulmonaryepithelioidhemangioendothelioma,PEH）是罕见的交界性的来源于肺内血管的肿瘤。现就本院收治的1例肺上皮样血管内皮瘤,结合相关文献进行讨论。     

Benign and malignant vascular tumors of the liver in adults

Vascular tumors of the liver in adult patients include cavernous hemangioma, a common benign tumor; epithelioid hemangioendothelioma, a rare, usually low-grade malignant tumor; and angiosarcoma, a rare and very aggressive tumor. All these primary mesenchymal tumors develop on a normal liver and may also affect other organs. Their pathogenesis remains largely unknown. Hepatic tumors are increasingly detected incidentally due to widespread use of modern abdominal imaging techniques. Therefore, reliable noninvasive characterization and differentiation of such liver tumors is of major importance for clinical practice. Hemangioma follows a benign course, and a nonoperative approach for the majority of these lesions is recommended. A definitive diagnosis of epithelioid hemangioendothelioma and angiosarcoma requires histopathologic examination. Liver transplantation at an early stage has greatly improved the prognosis of epithelioid hemangioendothelioma. The prognosis of angiosarcoma remains dismal. Designing a worldwide database that contains all data about patients with these rare diseases is recommended.     

Recurrence of pulmonary intravascular bronchoalveolar tumor with mediastinal metastasis 20 years later

Pulmonary intravascular bronchoalveolar tumor (IVBAT) also recognized as pulmonary epithelioid hemangioendothelioma, is a rare malignant vascular tumor of unknown etiology. IVBAT is a tumor of multicentric origin and the lungs are rarely involved, with only about 60 cases of pulmonary IVBAT described in the literature. The prognosis is unpredictable, with life expectancy ranging from 1 to 15 years. We report an unusual case of pulmonary IVBAT that recurred in the lung with metastasis to the mediastinum.     

肺上皮样血管内皮细胞瘤临床及病理特征

目的 分析3例肺上皮样血管内皮细胞瘤(PEH)的临床、影像及病理学特征,以提高对该病的认识.方法 回顾性分析3例PEH患者的临床、影像、病理学特征及免疫组织化学检查特点,并复习相关文献.结果 PEH临床表现较少且症状轻微,缺乏明显特征性,多表现为咳嗽、胸闷及活动后气促等.影像学表现为双肺多发弥漫性小结节影,病理学表现多呈结节状分布,细胞形态以上皮样或梭形细胞为主,瘤细胞胞质丰富,呈巢团状不规则排列,结节周边细胞较丰富,间质黏液变.瘤细胞一般缺乏多形性,核分裂及坏死少见.免疫组织化学染色CD31、CD34阳性,广谱角蛋白偶见灶性阳性,其他抗体阴性.目前尚无有效治疗措施,预后介于良、恶性之间.结论 肺上皮样血管内皮细胞瘤为一种上皮样血管内皮细胞分化的低度恶性肿瘤,临床症状不典型,易误诊为其他肺疾病,但具有独特的病理学特征,需要引起重视.

Abstract：

Objective To report 3 cases of pulmonary epithelioid haemangioendothelioma(PEH)and therefore to improve the understanding of this tumor. Methods The clinical pathological features of 3 cases of PEH were described and related literatures were reviewed. Results The etiology of this rare disease remained unknown. Symptoms were scanty and usually mild. Chest radiograph or computed tomography usually revealed multiple bilateral pulmonary nodules. Histologically, crown-like clusters of epithelioid tumor cells or spindle cells were filled in the alveoli at the periphery of the tumor nodules, while the central part of the nodules contained myxoid to hyaline matrix. Tumor cells generally lacked pleomorphism, mitotic activity and necrosis. They were immunohistochemically positive for CD31 and CD34.CK staining was positive in some cases. There was no effective treatment for this disease and its prognosis was unpredictable. Conclusions PEH is a low grade malignancy and represents a distinct clinical pathological entity. It is rare and often misdiagnosed as other pulmonary diseases.     

Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature

We describe a case of pulmonary epithelioid hemangioendothelioma, previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made by transbronchial biopsy. This is a rare disease, with approximately 50 cases described in the literature. All previous cases have been diagnosed by surgical lung biopsy. Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made on transbronchial biopsy; to our knowledge, this has not been previously described in the English-language literature. We also described findings on high-resolution CT, both typical and atypical relative to previously published reports. This tumor can affect multiple organs. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported.     

A case of primary hepatic epithelioid hemangioendothelioma mimicking metastatic carcinoma]

Epithelioid hemangioendothelioma is a rare vascular origin tumor which usually occurs in soft tissues, liver, and lung. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It is difficult to diagnose and treat because of non-specific clinical manifestations and findings on the imaging study. Moreover, pathological misdiagnosis is common. We report a case of this rare tumor that was detected incidentally. Final diagnosis was based on histological evidence. A 52-years old man suffered from right upper quadrant abdominal pain for 3 months, and was initially misdiagnosed as a metastatic carcinoma. Physical examination revealed superior cervical lymphadenopathy with mild hepatomegaly. Finally, hepatic epithelioid hemangioendothelioma was diagnosed on the basis of positive immunohistochemical staining for factor VIII, CD34, and VEGF. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.     

Alveolar hemorrhage with epithelioid hemangioendothelioma. A previously unreported manifestation of a rare tumor

Epithelial hemangioendothelioma (EH), originally termed intravascular sclerosing bronchioloalveolar tumor (IVBAT), is a rare pulmonary vascular neoplasm usually associated with an indolent but slowly progressive clinical course. We describe a patient with EH who presented with alveolar hemorrhage and died within 8 wk. This report extends the clinical spectrum of epithelial hemangioendothelioma to include this aggressive form. EH should be considered in the differential diagnosis of alveolar hemorrhage syndrome.