肺上皮样血管内皮瘤临床病理观察

目的 探讨肺上皮样血管内皮瘤的临床病理特点。方法 4例肺上皮样血管内皮瘤,3例女性,1例男性,年龄28～40岁,无自觉症状或有轻度咳嗽、气短。肺活检或手术切除标本经甲醛固定,石蜡包埋,常规HE及免疫组织化学(Envision法)染色。所用抗体包括CD31、CD34、细胞角蛋白(AEl／AE3)、TTF-1、波形蛋白和上皮膜抗原。结果 本组肺上皮样血管内皮瘤病例女性多于男性,胸部CT显示双肺多发弥漫性小结节影。病理形态特点为结节周边上皮样肿瘤细胞呈花冠状充填于肺泡腔,病变中心为黏液透明样变间质,肺泡壁结构保留,肿瘤细胞胞质内有空泡形成,空泡内偶见红细胞,肿瘤细胞异型性不明显,核分裂和坏死均少见,免疫组织化学染色示CD31、CD34阳性,AE1／AE3偶见灶状阳性,其他抗体呈阴性。结论 肺上皮样血管内皮瘤是一种具有独特临床病理特点的低度恶性血管来源肿瘤。     

肺上皮样血管内皮瘤1例

患者男性,45岁.1个月前感冒后出现全身乏力、咽部不适、鼻塞、流涕、打喷嚏、痰中带血块等症状.入院前4天患者晚上自觉发热,测体温37 ℃.CT及增强CT检查示左肺上叶结节灶,双下肺纹理重,肺癌不能除外(图1).在本院做痰培养,抗酸杆菌染色阴性.临床诊断为左肺上叶结节性质待查.     

上皮样血管内皮瘤的临床病理分析

目的：探讨上皮样（组织细胞样）血管内皮瘤的临床病理学特点及其意义。方法：对9例上皮样血管内皮瘤进行光镜和免疫组织化学SP法检测,1例作电镜观察,结果：年龄16－47岁,平均32岁,男女性别差异无显著性意义。部位：头面部4例,上肢3例,下肢2例,形态特征：瘤细胞具有上皮样或组织细胞样的形态;瘤细胞圆形或多角开,三五成群呈小巢状,索状,不规则状排列,分布于粘液间质中;间质可显著或少量粘液样变或玻璃样变,瘤细胞内含有原始血管腔,核分裂角,多形性及坏死少见,部分病例伴有梭形细胞血管内皮瘤改变,1例见破骨细胞样的多核巨细胞,免疫组织化学检测7例,7例波形蛋白均阳性,5例第八因子相关抗原,CD31,CD34阳性,2例细胞角蛋白弱阳性,1例CD68和a1-抗胰蛋白酶阳性,电镜观察1例见胞质内含丰富的微丝和少许的W－P小体,6例随访－8年,3例在原发部位有1或2次复发,但未见有转移,结论：上皮样血管内皮瘤是一种低度恶性的软组织肉瘤,其病因学尚不清楚,熟悉其形态特征对避免误诊为其他类似病变具具有重要意义。     

肝脏上皮样血管内皮瘤临床病理观察

目的 探讨肝脏上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理特点和鉴别诊断要点.方法 结合文献对3例肝脏EHE的临床资料、病理特征、免疫表型及治疗预后进行分析.结果 3例均为女性,年龄分别70、75和36岁.2例为肝脏内多发,1例为单发.典型的组织学特征表现为短梭形上皮样细胞伴多量胶原化的纤维间质,肿瘤细胞形成网眼状结构,并可见印戒样细胞,胞质内可包含单个红细胞.肿瘤呈浸润性生长,延肝窦及肝内小血管生长.免疫表型:vimentin(+++)、FⅧRAg(+++)、CD34(++)、CD31(++)、D2-40(++).而CK、CK7、CK8/18、Hep-1、AFP均(-).结论 肝脏EHE是一种罕见的肿瘤并且预后有差别,部分病例可迅速死亡.D2-40在肿瘤中有一定的阳性表达,是鉴别诊断中有价值的标记物.     

骨上皮样血管内皮瘤临床病理学观察

目的 探讨骨上皮样血管内皮瘤的临床病理特征和诊断。方法 对3例骨上皮样血管内皮瘤进行临床资料分析、光镜观察和免疫组织化学检测，并结合文献进行讨论。结果 3例病变部位均为下肢。最常见的临床症状是局部疼痛。X线表现为溶骨性骨破坏，1例伴有病理性骨折。组织形态学特征是上皮样瘤细胞形成较原始的小血管腔，呈巢状、索状、不规则形分布于有黏液样变或透明变性的间质中，肿瘤组织内或边缘散布成熟的骨小梁组织。3例肿瘤均表达vimentin、FⅧRAg和CD34。随访结果2例未见肿瘤复发，1例失访。结论 原发于骨的上皮样血管内皮瘤是较少见中间型血管源性肿瘤，其组织形态学要与骨上皮样血管瘤、上皮样血管肉瘤和转移性癌等鉴别。     

肺上皮样血管内皮瘤一例

患者男 ,6 4岁。 2d前无明显诱因出现咯血 ,气促。于2 0 0 1年 8月 2 7日入院。查体 :患者一般状态良好 ,全身浅表淋巴结无肿大 ,皮下及软组织未触及肿块。胸片示 :胸廓对称 ,气管略右偏 ,右肺门上部见一团块状高密度影 ,约 6 5cm× 5 5cm ,边界不清楚 ,两肺见弥漫分布的大小不等结节样高密度影。考虑为右肺癌两肺转移。CT示 :两肺可见散在分布大小不等小结节状阴影 ,密度中等 ,边界尚清 ,两侧肺门增大不明显 ,纵隔无增宽。考虑为右肺癌肺内转移。手术所见 :右胸膜腔有少量纤维膜样黏连 ,分离后见右肺中叶全部呈肝样实变呈紫黑色 ,肺内见…     

三脑室底上皮样血管内皮瘤1例临床病理分析

目的探讨颅内上皮样血管内皮瘤(epithelioid hemangioendothelioma, EHE)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例颅内EHE的临床病理特点、免疫表型等,并复习相关文献。结果患者男性,59岁,临床表现为左眼失明,肿瘤位于三脑室底,大小1.5 cm×1 cm×0.6 cm;镜下见肿瘤细胞呈上皮样及梭形,胞质丰富、嗜酸性,部分瘤细胞内含明显空泡,呈"印戒"状,内可见红细胞,间质胶原丰富,肿瘤局部侵犯周围脑组织。免疫表型:肿瘤细胞血管内皮细胞标志物CD31、CD34和vimentin均阳性;CKpan局灶阳性,S-100、EMA、PR、GFAP、CD68和TFE-3均阴性。结论颅内EHE是一种少见的低度恶性血管肿瘤,易误诊,应与转移癌、脊索瘤、脊索样脑膜瘤及脊索样胶质瘤等相鉴别。     

胸膜上皮样血管内皮瘤3例临床病理分析

上皮样血管内皮瘤(epithelioid hemangioendothelioma，EH)是一种特殊类型的上皮样、组织细胞样和其混合形态的血管内皮细胞源性恶性肿瘤。该瘤WHO(1994年)分类曾称交界性或中间性的血管内皮瘤，根据WHO(2002年)新分类现被列入软组织肿瘤中恶性的脉管肿瘤。该瘤罕见，组织形态结构多变，极易误诊，现报道3例。     

肾脏上皮样血管内皮瘤1例及文献复习

目的 探讨肾上皮样血管内皮瘤(epithelioid hemangioendothelioma, EHE)的临床病理学特征、诊断及鉴别诊断方法.方法 通过HE染色和免疫组化观察1例肾原发性上皮样血管内皮瘤并复习文献.结果 组织学上肿瘤由排列呈巢状和条索状的上皮样细胞组成,胞质丰富、嗜酸性,常见胞质内腔隙,部分腔隙内有红细胞,有些细胞核偏位,类似印戒细胞样,间质为黏液样.免疫表型:CD34、CD31和vimentin阳性,显示细胞内空泡为原始血管腔.结论 EHE是一种不常见的生物学行为介于血管瘤和普通型(高级别)血管肉瘤之间的血管肿瘤,其病理形态具有一定的特征性,发生在肾脏者罕见,其形态学和免疫表型与发生于肾外者相同.     

多部位发生的上皮样血管内皮瘤2例报道及文献复习

目的 探讨多部位发生的上皮样血管内皮瘤(EHE)的临床病理学特征.方法 回顾性复习2例多部位发生的血管内皮瘤的临床资料、组织学形态和免疫学表型,并复习文献.结果 2例均为女性,年龄分别为24和37岁,CT示双肺及肝弥漫结节影.病理学检查发现肝和肺结节的镜下形态相同,即在黏液样的间质背景中可见条索样、小巢状或单个散在分布的上皮样瘤细胞,瘤细胞有明显异型性,伴胞质内管腔形成,腔内偶见红细胞.免疫组织化学染色显示:瘤细胞表达内皮标记(CD34、CD31和F8),不表达CK、CD68、ER、PR.结论 上皮样血管内皮瘤是一种少见的低度恶性血管肿瘤,多部位同时发生者容易被误诊.上皮样血管内皮瘤的临床表现和生物学行为因其发生部位不同而有较大的差异.     

Pulmonary epithelioid hemangioendothelioma mimicking mesothelioma

An intrathoracic mass was discovered on magnetic resonance imaging (MRI) of the spine in a 37-year-old Caucasian man with a 1 year history of progressively severe upper back pain. A subsequent chest CT scan indicated a 4 cm left hilar mass, extending to the apex and encasing a portion of the left bronchus and pulmonary artery. Initial bronchoscopic and transthoracic biopsies failed to obtain diagnostic material. The patient underwent thoracotomy and was found to have a locally advanced, surgically unresectable lung tumor, involving the pleura, pericardium and diaphragm. The patient failed to respond to radiochemotherapy, and died 11 months following the diagnosis with tamponade and metastasis to the skin of the thoracoabdominal wall. Histologically the tumor had an epithelioid and spindled appearance, without high-grade histological features, and was initially thought to represent biphasic diffuse malignant mesothelioma. Positive immunohistochemistry for vascular markers (CD31, CD34, and FLI-1) disclosed the vascular nature of the tumor. Mesothelioma markers were universally negative and cytokeratin was focally reactive only in some epithelioid cells. Epithelioid hemangioendothelioma is a rare tumor in the lung that can mimic other more common pathological entities, and should be included in the differential diagnosis of unusual pulmonary neoplasms with epithelioid or biphasic morphology.     

A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology

Malignant epithelioid hemangioendothelioma (MEHE) is a rare vascular tumor with a biological behavior that lies between those of classical epithelioid hemangioendothelioma and angiosarcoma. Furthermore, MEHE is rarely diagnosed by fine needle aspiration cytology. The authors describe the cytological features of MEHE in a 41-year-old man who presented with increasing dyspnea over a period of 1 month before admission. Computed tomography of the chest showed a 3 cm poorly defined mass in the right lower lobe. Fine needle aspiration cytology demonstrated cellular smears of loosely cohesive clusters of epithelioid cells with numerous intracytoplasmic lumens in a necrotic background. Cellular features included fine chromatin and vesicular or slightly hyperchromatic nuclei with inconspicuous nucleoli and intranuclear inclusions. Nuclear membranes were relatively irregular with indentation. Mean N/C ratio was not increased, presumably due to a moderate amount of cytoplasm. The histologic examination displayed epithelioid and spindle cell proliferation with necrosis accompanying a classical epithelioid hemangioendotheliomatous area. The immunohistochemical evaluation was confirmatory and showed immunoreactivity for vascular markers. The authors also reviewed FNAB findings of epithelioid angiosarcoma, primary adenocarcinoma, and bronchioloalveolar carcinoma of the lung to identify cytomorphologic differences by literature bases. MEHE of the lung is difficult to diagnose cytologically because of its rarity and its cytomorphologic similarities with other malignant epithelial and mesenchymal tumors. However, it may be possible to distinguish it from other entities when the possibility of this unusual vascular neoplasm is suspected and ancillary studies are supportive.     

Cytopathologic features of hepatic epithelioid hemangioendothelioma

We present the cytological features of hepatic epithelioid hemangioendothelioma (EH), which is considered to be a vascular proliferation of intermediate malignant potential. The case report concerns a 52-yr-old previously healthy man discovered to have multiple hepatic masses upon evaluation for abnormal liver function tests. Fine-needle aspiration demonstrated a neoplasm composed of interanastomosing epithelioid cells that contained intracytoplasmic lumens. Histologic sections, immunohistochemistry, and ultrastructural evaluation were confirmatory. Although hepatic EH is a rare tumor, its characteristic cytological, histological, and ultrastructural features permit a straightforward diagnosis. It is important to distinguish this entity from adenocarcinoma and angiosarcoma because long-term disease-free survival is possible, especially in the setting of orthotopic liver transplantation. Diagn. Cytopathol. 17:50–53, 1997. © 1997 Wiley-Liss, Inc.     

淋巴结原发上皮样血管内皮瘤1例及文献复习

目的 探讨淋巴结原发上皮样血管内皮瘤的诊断及鉴别诊断。方法 1例淋巴结原发上皮样血管内皮瘤进行HE切片、组织化学、免疫组化等观察，并复习文献进行讨论。结果 光镜下肿瘤细胞呈多角形或类圆形上皮样，无明显核分裂象，组织结构多种，呈单个细胞条索、实性巢状及管腔样结构排列，瘤细胞形成小的细胞内管腔，见胞质内空泡，并见红细胞，此特征为细胞原始管腔结构。玻璃样间质。网状纤维染色显示血管腔隙结构。肿瘤细胞表达Vim、CD31、FⅧRAg，少数细胞表达CD34。结论 淋巴结原发上皮样血管内皮瘤是极少见的低度恶性血管性肿瘤，可出现多种组织结构，有一定的特征性。应与转移性癌、转移性恶性黑色素瘤、上皮样血管肉瘤、上皮样血管瘤、上皮样肉瘤及硬化性上皮样纤维肉瘤等鉴别。     

骨上皮样血管内皮细胞瘤9例诊治分析

目的 探讨骨上皮样血管内皮细胞瘤(B-EHE)的诊断、治疗方法。方法 回顾性分析2002年2月—2015年9月解放军东部战区总医院骨科收治的9例B-EHE患者的临床资料。其中男6例、女3例,年龄15~64岁。术前X线及CT检查均表现为溶骨性改变,1例MRI可见周围软组织受累,1例出现肺部转移灶。3例选择病灶刮除、植骨内固定;1例腰椎病灶选择肿瘤切除椎板减压内固定;3例近关节病灶选择肿瘤切除假体置换,其中1例术后化疗;1例多发病灶伴肺转移选择活检确诊后化疗治疗;1例病理性骨折伴出血选择行截肢术后综合治疗。结果 9例中失访1例,其余随访时间8～72个月。现7例存活。3例行病灶刮除植骨内固定,2例局部复发,二次手术后治愈。1例腰椎肿瘤切除减压固定,术后恢复良好。3例行肿瘤切除假体置换术,1例失访;2例术后治愈,关节功能良好,其中1例术后辅助化疗,未出现局部复发和病灶转移。1例多发病灶伴肺部转移行化疗,肺部无进展,局部无进展。1例行截肢术后综合治疗者,肿瘤复发迁延性出血,最终因器官衰竭死亡。结论 B-EHE为低-中度恶性肿瘤,术前影像学无特异性,诊断困难,主要依靠术后病理结果诊断。治疗首选边界切除,辅以化疗或放疗,可降低局部复发率和远处转移。     

Pitfalls in the diagnosis of hepatic epithelioid hemangioendothelioma by FNA and needle core biopsy

Epithelioid hemangioendothelioma (EHE) of the liver is an extremely rare tumor that masquerades as an epithelial neoplasm and poses significant diagnostic pitfalls for the cytopathologist. This report describes a 64‐year‐old woman whom on computerized tomography was found to have multiple peripherally calcified hypodense lesions throughout the liver and an ipsilateral adrenal mass. Fine needle aspiration and needle cores biopsies of the largest liver lesion showed epithelioid cells and spindle cells in fibrous stroma. The epithelioid cells had round or oval, hyperchromatic nuclei with smooth nuclear contours, delicate cytoplasm and indistinct cytoplasmic borders. Rare cells had intracellular vascular lumen containing fragmented or intact red blood cells. The cells were individually dispersed or arranged in nests and vague tubular arrays. No mitotic figures or necrosis were seen. An immunohistochemical profile demonstrated diffuse CD31 and focal CD 34 and nuclear Fli‐1 immunoexpression and low Ki‐67 proliferative activity (1%) within lesional cells confirming the diagnosis of EHE. The differential diagnosis of EHE which includes cholangiocarcinoma, hepatocellular carcinoma, metastatic carcinoma and melanoma is discussed. Diagn. Cytopathol. 2014;42:516–520. © 2013 Wiley Periodicals, Inc.