上皮样血管内皮瘤9例临床病理分析

摘 要：［目的］ 分析上皮样血管内皮瘤的临床病理学特点,以提高诊断能力。［方法］ 回顾性总结9例手术切除或粗针穿刺活检诊断为上皮样血管内皮瘤的临床病理学特征及免疫组化表型。 ［结果］ 患者男性5例,女性4例;年龄16~63岁,平均年龄42岁。肿瘤分别位于肺4例、肝脏2例、软组织2例、输尿管1例。镜下观察肿瘤细胞形成上皮样形态,胞浆丰富嗜酸性,条索状或不规则散在排列,内含红细胞。间质为丰富的透明变性、黏液或黏液软骨样基质,有时可见钙化、骨化及坏死。免疫组织化学染色显示肿瘤细胞不同程度表达Vimtenin、CD31、CD34、D2-40、Fli-1、第八因子相关抗原,均未表达TFE3。8例获随访,随访时间为1~24个月均健在。［结论］ 上皮样血管内皮瘤,可见于多种实质性器官。由于其临床表现及影像学特征不典型,故不易早期发现;更因其组织形态类似于上皮样细胞,而易误诊为癌;应结合组织学特征和血管源性免疫组化表型及分子检测避免误诊。     

肺上皮样血管内皮瘤一例临床病理观察

目的 探讨肺上皮样血管内皮瘤的临床病理特征,提高对该病的认识.方法 对1例肺上皮样血管内皮瘤进行组织学观察及免疫组织化学标记,并复习相关文献.结果 镜下见瘤组织在肺内呈小结节状肾小球样增生,充满肺泡腔,局灶区域可见圆形、梭形的上皮样细胞区,胞质空泡形成.免疫组织化学标记肿瘤细胞表达CD34、CD31、Vimentin、CEA,而CK7、CK20、TTF-1、CK、间皮细胞、EMA均为阴性.结论 上皮样血管内皮瘤是一种低度恶性肿瘤,发生在肺内可形成多个小结节,应注意与上皮样血管肉瘤、肺腺癌、间皮瘤相鉴别.     

肺上皮样血管内皮瘤遗传学分析

背景与目的:初步探讨肺上皮样血管内皮瘤细胞遗传学改变的特点.材料与方法:提取2例肺上皮样血管内皮瘤石蜡包埋的肿瘤组织DNA,利用微阵列比较基因组杂交(array-CGH)技术检测瘤组织DNA拷贝数缺失和扩增片段.结果:2例肺上皮样血管内皮瘤DNA拷贝数改变有诸多类似之处,且均以多条染色体DNA小片段(小于10 Mb)扩增为主,很少有缺失区域;具体涉及的基因分别为150个和556个,共同改变的基因为77个.结论:肺上皮样血管内皮瘤细胞遗传学改变可能主要以小片段DNA扩增为特点,该特点可以诠释此肿瘤的交界性或低度恶性临床病理特征.     

大阴唇上皮样血管内皮细胞瘤1例

1 病案摘要 患者,女,27岁,因外阴肿物伴轻度疼痛一年就诊。妇科检查,右侧大阴唇内侧查见一肿块,体积5cm×3.5cm×2.5cm,质中,表面光滑,活动欠佳,轻压痛。术中所见:肿物位于皮下软组织,与周围组织粘连,剥离后完整切除。临床诊断:外阴囊肿。病理检查:扁圆形肿物1个,体积4.5cm×3cm×1.5cm,包膜不清,表面粘连,切面灰红色,质硬韧。镜下:瘤细胞以血管为中心呈离心性向周围增生。细胞圆形或多角形,胞浆丰富,淡嗜酸性,内有大小不等的空泡,有的空泡内含红细胞(细胞内原始血管腔),核圆呈泡状,核仁明显(图1);瘤细胞呈小巢状或条索状排列,分布于黏液样变性及玻璃样变性的间质中(图2);少部分细胞梭形,呈梭形细胞血管内皮瘤图像。细胞无明显异型性。特殊染色:黏液PAS阴性。免疫组织化学染色:瘤细胞波形蛋白、第八因子相关抗原、C-角蛋白均呈阳性,肌动蛋白、结蛋白阴性。病理诊断为右侧大阴唇上皮样血管内皮细胞瘤。     

肺上皮样血管内皮瘤的诊治进展

上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)属于血管源性肿瘤,起源于血管内皮,可发生于身体多个部位,以肝脏、骨骼和肺为主。而原发于肺的上皮样血管内皮瘤(pulmonary epithelioid hemangioendothelioma,P-EHE)是罕见的中低度恶性肿瘤,大多以散发病例报道为主,缺乏流行病学调查。因ＰP-EHE发病率低、临床症状及影像学表现缺乏特异性,易误诊、漏诊,导致患者接受不适当治疗及预后不良。其诊断依据是基于独特的组织学、免疫组化及分子特征。因P-EHE较为少见,目前尚无标准有效治疗措施,手术适用于病灶较少的病例,放疗在骨转移病灶及病灶局控上显示较好疗效,抗血管生成的靶向治疗显示较好的应用前景。P-EHE异质性较强,预后介于良性及高度恶性肿瘤之间。本文对该病的发病机制、诊断、临床特征、治疗及预后的研究进展进行综述。     

上皮样血管内皮细胞瘤4例临床病理分析

上皮样血管内皮细胞瘤( epithelioid haemangioendothelioma, EHE) 亦称为组织细胞样血管内皮瘤,多见于成年女性,可发生于肝脏、皮肤、肺、淋巴结等全身多部位. 肿瘤由排列成巢的上皮样或组织细胞样内皮细胞构成,具有特殊的玻璃样或软骨样间质成分. EHE是1种以血管为中心的中间型肿瘤,具有转移潜能[1]. 因此我们搜集4 例 EHE患者的临床资料,共同讨论EHE临床表现预后及病理组织学特点.     

上皮样血管内皮瘤5例临床病理学观察

上皮样血管内皮瘤(ep ithelio id hem ang ioendo-theliom a,EHE)是一种起源于血管较少见的软组织肿瘤。临床及病理易误诊为癌。本文结合文献对该肿瘤的临床病理、免疫组化特征及鉴别诊断进行讨论。1材料与方法收集本院1995年1月~2003年12月上皮样血管内皮瘤存档标本5例,其中男     

肝脏和肺内上皮样血管内皮细胞瘤的临床病理观察

目的　探讨肝脏和肺内上皮样血管内皮细胞瘤（ＥＨＥ）的临床病理特点。方法　收集３例ＥＨＥ患者的临床病理资料并复习相关文献,分析该病的临床病理特征。结果　３例患者均为女性,其中肺ＥＨＥ１例,肝脏ＥＨＥ２例。典型病理表现为脏器多发性、边界不清的小结节;瘤组织呈多结节状;瘤细胞呈卵圆形或圆形上皮样,胞浆丰富嗜酸性,间质黏液样变性或透明变性,胞浆内有大空泡,空泡内偶见红细胞。免疫组化染色示Ｆ８、ＣＤ３１、ＣＤ３４为阳性表达。结论　ＥＨＥ是一种低度恶性血管源性肿瘤,易误诊,诊断主要依靠病理形态学和免疫组化染色。     

肝上皮样血管内皮瘤的临床病理观察

目的 探讨肝上皮样血管内皮瘤（EH）的临床病理特点、鉴别诊断及预后。方法 观察4例肝EH的组织形态学特点并行免疫组化检测,并结合相关文献进行分析。结果 EH镜下肿瘤细胞为上皮样、梭形或多角形,呈条索状、小巢状生长,或在血管内呈乳头状生长,部分瘤细胞胞质空泡化,内含红细胞;核分裂像罕见,瘤细胞胞质丰富,嗜酸性,胞界不清,间质黏液样变或玻璃样变。免疫组化染色显示肿瘤细胞CD31、CD34、Vim阳性。结论 肝EH是一种少见的低度恶性的血管源性肿瘤,影像学易误诊,需依靠病理形态学及免疫组化确诊。     

Pulmonary Epithelioid Hemangioendothelioma: A Case Report

A 45-year-old woman whose x-ray film revealed multiple widespreadbilateral small nodules was diagnosed as having pulmonary tuberculosisor metastatic pulmonary cancer. A conclusive histological diagnosisof pulmonary epithelioid hemangioendothelioma (PEH) was madefollowing an open-lung biopsy. PEH is a progressive diseasethat probably originates from an endothelial cell. However,there is still no effective therapeutic modality for PEH. Thepostoperative course of our patient has been uneventful withno evidence of tumor growth for four and a half years afterinitial x-ray detection of the lesions.     

Malignant Intracranial Epithelioid Hemangioendothelioma Presumably Originating from the Lung: Case Report

OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. Magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.     

肺淋巴管平滑肌瘤病的临床病理分析

背景与目的肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)是一种罕见的具有独特临床病理特征的肿瘤性疾病,本文旨在探讨PLAM的临床病理学特点及其鉴别诊断。方法收集3例PLAM病例,复习患者的临床资料,进行组织学及免疫组化观察,并结合文献复习分析。结果 3例PLAM病例均为育龄妇女,年龄27岁-45岁,平均年龄37.7岁,其中2例胸部CT示双肺弥漫分布薄壁的含气囊腔,1例为气胸。组织学检测显示肿瘤组织呈不规则腔隙和斑片或结节状,由内皮细胞及其周围大量增生的平滑肌样梭形细胞构成,可沿支气管、血管等分布。免疫组织化学检测示增生平滑肌样梭形细胞呈现结蛋白(Desmin,Des)、钙结合蛋白(Caldesmon,Caldes)、平滑肌肌动蛋白(α-Smooth muscle actin,SMA)、肌特异性肌动蛋白(Muscle-speci c actin,MSA)、人类黑色素瘤单克隆抗体(HMB-45)、黑色素瘤标记物(Melanoma Marker,CD63)、波形蛋白(Vimentin,Vim)、雌激素(Estrogen,ER)和孕激素(Progestogen,PR)阳性表达,而T细胞1识别的黑色素抗原(Melanoma Antigen Recongnizedby T cell1,M T-1)为阴性表达,毛细血管内皮细胞第8因子(FVIII)、人类造血干细胞表面分子(CD34)阳性表达,淋巴管内皮细胞呈淋巴管内皮细胞标记物(D2-40)阳性表达。本组病例随访时间为3个月-25个月,生存状况良好,未见该病复发,除肺外其余各系统未见淋巴管平滑肌瘤病等。结论 PLAM的主要病理改变为沿支气管、血管分布的淋巴管周围平滑肌细胞异常增生,一般发病于绝经期前育龄女性,渐进性呼吸衰竭导致该病预后不良。     

11例上皮样血管肉瘤的临床病理分析

[目的]探讨上皮样血管肉瘤的临床病理学特征及诊断、鉴别诊断要点。[方法]分析1995～2007年我院收治的11例上皮样血管肉瘤临床病理资料。[结果]镜下观察肿瘤细胞弥漫成片或聚集成巢，伴有出血和多灶性坏死。瘤组织内常见互相吻合、大小不等的不规则腔隙，其内衬覆异型瘤细胞，可见单个或多个瘤细胞形成空腔包绕红细胞。瘤细胞9例CD34阳性、10例CD34阳性。术后存活期5周～55个月。[结论]上皮样血管肉瘤是血管肉瘤的一种特殊类型，免疫组织化学检测有助于确定肿瘤细胞的内皮分化。     

Epithelioid hemangioendothelioma of the liver: Etiology, natural history and treatment

Introduction Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor, which also has been called histiocytoid haemangioma. This low-grade malignant tumor was first described as a distinctive entity in 1982 by Weiss and Enzinger[1]. Histopathologically, EHE is charac-terized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation[2]. The tumor may occur in numerous organs, includingliver, lung, parotid grand, skin, pleura, thyroid, spleen, cardiac valve,radial artery, oral cavity and parapharyngeal space[2-11]. Many cases have been reported, but its etiology and natural history remain un-clear.     

骨上皮样血管内皮瘤2例并文献复习

目的:探讨2例骨上皮样血管内皮瘤（epithelioid hemangioendothelioma,EHE）的临床特点、治疗措施和预后因素。方法:回顾性分析2例原发于骨的EHE临床资料、诊断、治疗以及随访情况,并结合文献进行讨论。结果:2例骨EHE中男性和女性各1例,低度恶性的单发骨EHE患者经过局部病灶的放疗、TE方案联合恩度等综合治疗,目前仍存活;侵袭性多发病灶的骨EHE患者经过TE方案联合恩度系统治疗、局部TOMO等综合治疗,病情进展后死亡。结论:骨EHE的预后主要取决于肿瘤组织学级别和发生的部位;综合治疗可适当延长生存期,规范治疗尚无循证医学依据。     

Activity of Angiogenesis Inhibitors in Metastatic Epithelioid Hemangioendothelioma: A Case Report

This report describes a patient with metastatic epithelioid hemangioendothelioma treated with bevacizumab and nanoparticle albumin-bound paclitaxel.The treatment was well tolerated and led to the stabilization of an aggressive variant of the disease. This case report is the first one that describes the activity of the combination of chemotherapy and bevacizumab in epithelioid hemangioendothelioma.Literature describing the activity of bevacizumab and other agents(thalidomide,lenalidomide,and interferon) believed to possess anti-angiogenic activities is also reviewed.