胸膜上皮样血管内皮瘤误诊1例

上皮样血管内皮瘤(epitthelioid hemangioendothelioma,EHE)是一种罕见的恶性血管肿瘤,胸膜上皮样血管内皮瘤(PEH)发病率更低,且临床症状和体征多不典型,极易误诊,现将我科收治的1例报道如下。     

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。     

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一例并文献复习

目的 提高对肺上皮样血管内皮瘤的认识。方法 结合1例肺上皮样血管内皮瘤患的临床资料和献复习，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；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，患预后介于良、恶性肿瘤之间。结论 肺上皮样血管内皮瘤罕见，极易误诊，应予重视。     

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。     

结节性甲状腺肿合并肺上皮样血管内皮瘤误诊为甲状腺癌一例

临床资料 结节性甲状腺肿好发于女性，发病率很高，临床上往往与甲状腺癌鉴别困难。肺上皮样血管内皮瘤（pulmonary epithelioidhemangioendotheliom，PEH）是一种罕见的肺内低度恶性血管源性肿瘤，     

肺上皮样血管内皮瘤的CT特征及临床分析

目的 探讨肺上皮样血管内皮瘤(PEH)的CT影像特征及临床特点.方法 回顾性分析2014年1月至2020年11月就诊于我院经病理证实的4例PEH患者的临床及CT影像资料.结果 PEH患者4例均为男性,平均年龄45.8岁;2例无吸烟史、2例偶有吸烟史.初诊时有咳嗽、咳痰1例,右侧胸痛1例,无症状经体检发现2例.病理学免疫...     

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.     

Case report of pulmonary epithelioid hemangioendothelioma]

A 45-year-old man was admitted to our hospital because of multiple nodular shadows in the right upper field of a chest radiogram taken at a regular medical checkup. He underwent open lung biopsy. The lung tumor found was diagnosed histologically as pulmonary epithelioid hemangioendothelioma. The tumor cells showed positive staining for CD34 and factor VIII-related antigen. Pulmonary epithelioid hemangioendothelioma (PEH) is a rare lung tumor, of which only 40 cases, including the present case, were reported between 1983 and 2002 in Japan. PEH is a progressive, low-grade malignant tumor that originates from hemangioendothelial cells. In chest radiography or CT scanning, PEH is usually discovered incidentally as multiple nodular shadows. Many cases of PEH are diagnosed by open lung biopsy or thoracoscopic biopsy. No standard therapy for PEH has yet been established, other than resection of a solitary lesion. The present patient has been followed without treatment for five-and-a-half years, and is still alive with no symptoms.     

A case of pulmonary epithelioid hemangioendothelioma detected on medical checkup]

A 51-year-old woman was admitted to our hospital because of bilateral multiple nodular shadows detected on a medical checkup chest X-ray film. Video-assisted thoracoscopic biopsy and hematoxylin eosin staining showed protrusion of the polypoid tumor tissue into the alveolar lumina, and the centers of nodules were occupied by hyalinized matrices. Immunohistochemical stain demonstrated that tumor cells were positive for factor VIII-related antigen, CD-34, and vimentin. The tumor was therefore diagnosed as pulmonary epithelioid hemangioendothelioma (PEH). PEH usually grows slowly. Standard therapy for PEH has not yet been established. We have followed the patient for 2 years without any treatment, but no symptoms have appeared.     

Pulmonary epithelioid hemangioendothelioma with PlGF expression: report of a case

Pulmonary epithelioid hemangioendothelioma (PEH) is a relatively uncommon neoplasm of vascular origin with a low or intermediate grade of malignancy. We present a case of a 28-year-old female with multiple pulmonary nodules which were diagnosed as PEH by video-assisted thoracoscopic surgery (VATS) biopsy. In addition, we performed an immunohistochemical analysis for placenta growth factor (PlGF) and a strong positivity for PlGF observed, suggesting that the PlGF may play some role in the tumorigenesis of PEH.     

Risk factors and independent predictors of survival in patients with pulmonary epithelioid haemangioendothelioma. Review of the literature and a case report

Pulmonary epithelioid haemangioendothelioma (PEH) is a rare pulmonary neoplasm. A patient with PEH with lymph node and pleural metastases that were discovered incidentally is described. An abnormal left upper lobe shadow was noticed on CXR in a 70-year-old woman during an assessment for the sudden onset of nausea and vomiting. Transbronchial lung biopsy did not provide a diagnosis. Lobectomy and lymph node resection were performed. The histological diagnosis of PEH was confirmed immunohistochemically by positive reactions to factor VIII-related antigen and CD34. Data on 93 patients with PEH including the present case report were analysed by Cox regression analysis using forward stepwise method to identify the risk factors, and the independent predictors of survival in patients with PEH. It revealed that male, symptomatic patients, presence of cough, haemoptysis, chest pain, multiple unilateral nodules, pleural effusion, metastases to more than one site and lymph node metastases were all significant risk factors for PEH (P<0.05). Symptomatic patients and presence of pleural effusion were the independent predictors of survival in patients with PEH.     

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。     

Hepatic epithelioid hemangioendothelioma treated with orthotopic liver transplantation: a case report.

Hepatic epithelioid hemangiendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patients condition. Orthotopic liver tranplantation is the choice of treatment in diffuse cases without metastases. A 32 year old woman was admitted to hospital with multiple mass lesions diagnosed by ultrasonography of the liver. Physical examination was normal except for a painless hepatomegaly, and her biochemical tests were within the normal range. Computed tomographic scanning showed the presence of multiple lesions in both lobes, some of which were accompanied by a small degree of calcification. Although these findings were suggestive of hepatic epithelioid hemangioendothelioma, ultrasonographic guided fine needle aspiration biopsy failed to diagnose the exact nature of the lesions. The diagnosis of hepatic epithelioid hemangioendothelioma was confirmed by diagnostic laparotomy and immunohistochemical examination of the specimen by FVIII-RAg, CD34 and CD 31 markers. The patient was treated by orthotopic liver transplantation and had no evidence of tumor 18 months after transplantation. The problems in differential diagnosis and treatment options are discussed in this report of the first case of this rare tumor, treated by orthotopic liver transplantation in Turkey.     

Epithelioid hemangioendothelioma associated with nodular regenerative hyperplasia

Epithelioid hemangioendothelioma of the liver is a rare neoplasm of vascular origin. We report a case of epithelioid hemangioendothelioma occurring in a patient with nodular regenerative hyperplasia. This association suggests that some hepatic vascular changes might promote the growth of epithelioid hemangioendothelioma.