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。     

肺上皮样血管内皮瘤的临床病理观察

目的探讨肺上皮样血管内皮瘤(pulmonaryepithelioidhemangioendothelioma,PEH)的临床病理特征、诊断及鉴别诊断。方法对3例PEH进行光镜观察和免疫组化标记,并结合文献进行分析。结果镜下见肿瘤细胞具有上皮样或组织细胞样形态,瘤细胞为圆形或多角形,呈小巢状、索状甚至腺样结构或不规则状,分布于黏液间质中;间质可见少量黏液样变或玻璃样变;瘤细胞内含有原始血管腔,核分裂象、多形性及坏死少见。肿瘤细胞表达CD34、FⅧRAg、CD31等血管内皮细胞标记,部分病例同时表达CK和(或)Vim。结论PEH是一种低度恶性肿瘤,其病理形态具有一定的特征性,诊断时需要与肺转移癌、肺上皮样血管肉瘤、肺淋巴管肌瘤病鉴别。     

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.     

上皮样肉瘤样血管内皮瘤的临床病理学观察

目的 探讨上皮样肉瘤样血管内皮瘤的临床病理学特征和鉴别诊断.方法 回顾性分析3例上皮样肉瘤样血管内皮瘤的临床表现、组织学特点和免疫表型.结果 3例均发生于成年男性,分别因左颈部肿块、髂部疼痛和双侧颈肩区复发性肿块就诊.镜下观察肿瘤由梭形和上皮样的细胞组成,两种细胞在形态上有移行.瘤细胞呈片状、模糊结节状或交织条束状排列,间质伴有胶原化.1例于结节中央可见凝固性坏死,形态上类似上皮样肉瘤.3例肿瘤内均无明显的血管形成,但其中1例于局灶区域可见胞质内空泡形成,类似上皮样血管内皮瘤.免疫组织化学标记,瘤细胞同时表达上皮性标记和内皮标记.3例均经手术切除,其中2例患者术后恢复良好,随访18个月和14个月均健在,无局部复发或远处转移,另1例术后6年内复发5次.结论上皮样肉瘤样血管内皮瘤属于一种少见的中间型血管内皮瘤,兼具上皮样肉瘤和上皮样血管内皮瘤的部分形态.仅凭光镜形态有时较难确定其内皮细胞分化,必须借助于免疫组织化学标记.上皮样肉瘤样血管内皮瘤与上皮样血管内皮瘤关系较为密切,可能是后者的一种富于细胞性梭形细胞变型.

Abstract：

Objective To study the clinicopathologic features and differential diagnosis of epithelioid sarcoma-like hemangioendothelioma (ES-H). Methods The clinical, radiologic and pathologic features of three cases of ES-H were analyzed. Results All the 3 cases occurred in male adults. The age ranged from 44 to 53 years. The presentations included left neck mass, iliac pain and bilateral shoulder masses. Histologically, ES-H was composed of a mixture of spindle and epithelioid tumor cells. Transition between the two cell types was demonstrated. The tumor cells were arranged in compact sheets, vague nodules or intersecting fascicles, amongst a collagenous stroma. Central coagulative necrosis was identified in one case, reminiscent the morphology that seen in epithelioid sarcoma. There was no evidence of angiogenesis, though focal presence of intracytoplasmic vacuoles was seen in one case, as in classic examples of epithelioid hemangioendothelioma. Immunohistochemical study showed that the tumor cells expressed both epithelial (AE1/AE3, CAM5.2 and epithelial membrane antigen) and endothelial (CD31,Fli-1 and factor Ⅷ-related antigen ) markers. Two of the cases were also positive for CD34. All of the patients were treated by surgical resection. Two patients remain well at 14-month and 9-month follow up,respectively. The remaining patient had repeated local recurrences during a 6-year period. Conclusions ES-H represents a rare morphologic type of hemangioendothelioma. It has some overlapping histologic features with epithelioid sarcoma and epithelioid hemangioendothelioma. The endothelial nature of ES-H is difficult to be verified on the basis of morphologic examination alone. Confirmation of the diagnosis with immunohistochemistry is necessary. ES-H is likely related to epithelioid hemangioendothelioma and may represent a cellular spindie cell variant of epithelioid hemangioendothelioma.     

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.     

肝脏上皮样血管内皮瘤

1982年，Weiss等首次描述了上皮样血管内皮瘤(cpithelioid haemangioendothelioma，EHE)，该瘤的恶性程度具有不确定性，临床病程介于血管瘤和血管内皮肉瘤之间，好发于软组织、肺和骨，亦可见于脾脏、乳腺、心脏、头颈、脑脊膜和脑、胃肠道等部位。早在1975年，Dail等就曾描述过发生于肺部类似EHE的肿瘤，当时称之为血管内细支气管肺     

脑上皮样血管内皮瘤1例及文献复习

目的：探讨脑上皮样血管内皮瘤（ＥＨＥ）的诊断及鉴别诊断。方法：通过ＨＥ、免疫组化及电镜观察１例脑原发性多灶性上皮样血管内皮瘤、并复习文献。结果：肿瘤由排列呈巢状、索状的上皮样细胞组成,胞浆丰富、嗜酸性;内含明显空泡呈“印戒”状,有的空泡内可见红血球,间质含粘液样基质。免疫组化染色显示ＦⅧ、ＣＤ３４、ＣＤ３１、ＵＥＡ－１和ｖｉｍｅｎｔｉｎ阳性;而ＣＫ、ＣＥＡ、ＥＭＡ、ＧＦＡＰ、Ｓ－１００蛋白及Ｍａｃ     

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.     

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。     

Epithelioid hemangioendothelioma of bone and soft tissue: A fine-needle aspiration biopsy study with histologic and immunohistochemical confirmation

We retrospectively reviewed two fine-needle aspiration biopsy (FNAB) specimens from two patients with histologically confirmed epithelioid hemangioendothelioma (EH). Both patients were men, ages 79 and 39 years; their primary tumors arose in the soft tissues of the mediastinum and within the proximal tibia, respectively. The former patient had symptoms of superior vena cava syndrome; multicentric intraosseous lesions involved the proximal tibia of the latter patient. All cytologic smears were hypercellular and composed of mostly disassociated single cells and small aggregates of ovoid to polygonal-shaped epithelioid cells. Nuclei were variable, ranging from ovoid and reniform to round and polylobated and surrounded by an abundant amount of dense cytoplasm. Binucleated epithelioid neoplastic cells were frequent. Nuclear pleomorphism ranged from slight to moderate, and small solitary to multiple nucleoli were identified within the majority of tumor cells. Rare neoplastic cells with a single, sharply demarcated intracytoplasmic vacuole and intranuclear cytoplasmic pseudoinclusions were observed in the smears of one tumor. Metachromatic stromal fragments, probably representing hyalinized chondromyxoid stroma, were seen in the other tumor. Neither case was recognized initially on FNAB as EH. Immunohistochemically, sections from the surgical biopsy specimens of both cases showed diffuse and strong immunopositivity for the endothelial marker CD31. Although the cytomorphology of EH appears distinct, clinicoradiologic correlation is essential, and immunohistochemistry may be helpful to avoid misdiagnoses. Diag. Cytopathol. 1998;19:38–43. © 1998 Wiley-Liss, Inc.     

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.     

骨上皮样血管内皮瘤4例及文献复习

目的探讨4例骨上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理特点、诊断和鉴别诊断。方法对4例原发于骨的EHE临床资料、组织学、免疫组化进行观察分析并随访,结合文献讨论。结果 4例骨EHE中男性和女性各2例,中位年龄24岁,发生部位包括右肩胛骨、腰4～5椎体、胫骨下段和左足。临床症状主要为疼痛,影像学资料提示溶骨性改变;镜下示上皮样的肿瘤细胞呈巢状、索状或小梁状排列,肿瘤细胞呈圆形、多边形间或梭形,常见明显的胞质内空泡并伴有周围间质的玻璃样或黏液样变性。免疫表型:瘤细胞CD31、CD34、α-SMA均阳性(100%),CKpan有1例阳性(25%),EMA均阴性(100%),肿瘤增殖标记Ki-67呈低表达。结论原发于骨的EHE是一种少见的低度恶性的血管源性肿瘤,它的诊断要结合临床和影像学资料,依据其病理学特征及相关的免疫组化特点综合判断,需与骨转移性癌、上皮样血管瘤、上皮样血管肉瘤(epithelioid angiosarcoma,EAS)等鉴别。     

上皮样血管平滑肌脂肪瘤7例临床病理分析

目的回顾分析上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的形态学特点、免疫表型、诊断及鉴别诊断、临床特点及预后,加深对EAML的认识,提高诊断的准确性。方法观察7例EAML的病理形态学、免疫表型及临床特征。结果 7例EAML形态相似,肿瘤细胞体积大,呈卵圆形、梭形或多边形,胞质丰富,嗜酸性,细胞核大,大小不一,核仁明显,可见多核及巨核瘤细胞,有少数核分裂象,呈巢、片状分布,部分围绕血管呈袖套状。免疫表型:肿瘤细胞HMB-45、Melan-A、vimentin、SMA均弥漫阳性,EMA、CK、CD10均阴性。7例EAML均行肿瘤根治性手术或肿瘤切除术,术后随访3~56个月,除1例因有高度恶性十二指肠间质瘤病史于3个月后死于恶性间质瘤进展外,其余6例均健在,多次复查B超、CT等均无复发、转移。结论 EAML是具有恶性潜能的间叶源性肿瘤,病理形态学上易误诊为恶性肿瘤,免疫组化对鉴别诊断有重要的意义,目前手术治疗是主要方法,有复发或转移可能。     

Pleural Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular origin. While it can be found in any tissue, it is most often found in lung and liver and usually has an intermediate behavior. EHEs originating from pleural tissue have been less frequently described than those from other sites. Furthermore, to date, all of the cited pleural EHEs were described as highly aggressive. In the present report, we describe a rare case of pleural EHE extending to lung and bone in a 31-year-old woman. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. Her disease stabilized during the 4th course of adriamycin (45 mg/m², day 1-3), dacarbazine (300 mg/m², day 1-3) and ifosfamide (2,500 mg/m², day 1-3) with mesna, and she survived for 10 months after the diagnosis.     

Epithelioid hemangioendothelioma in pleural effusion

Epithelioid hemangioendothelioma (EHE) is a rare mesenchymal tumor of vascular origin with variable morphological features and unpredictable biological behavior. EHE occasionally involves the pleural fluid. However, the cytomorphology of EHE found in pleural fluid has not been well characterized in the literature. Herein we describe a case of EHE, initially presenting as multiple liver lesions plus several small nodular densities at the bases of the lungs followed by bilateral pleural effusions. Diagn. Cytopathol. 2015;43:751–755. © 2015 Wiley Periodicals, Inc.     

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。     

假肌源性血管内皮瘤6例临床病理分析

目的 探讨假肌源性血管内皮瘤(pseudomyogenic hemangioendothelioma,PHE)的临床病理学特征、免疫表型、诊断及鉴别诊断.方法 对6例PHE的临床病理资料和免疫表型进行回顾性复习.结果 男性4例,女性2例.平均年龄32岁(10～70岁).3例原发于骨内,均为多骨性累及,其中1例伴发皮肤结节;另3例分别表现为软组织局部肿块、皮肤和软组织多灶性病变及皮肤黑色斑块.影像学检查:3例骨病变均表现为多骨性骨质破坏,1例皮肤和软组织多灶性病变累及多个组织平面.眼观:送检肿瘤组织呈灰白、灰红或灰褐色,直径0.5 ～4 cm,平均2 cm.镜检:病变边界不清,可累及真皮、皮下、肌肉或骨组织.肿瘤主要由疏松条束状、结节状或片状分布的胖梭形细胞组成,部分区域内瘤细胞呈上皮样,间质内可见散在的中性粒细胞.高倍镜下见瘤细胞胞质丰富,嗜伊红色,核染色质呈空泡状,可见小核仁,形态上类似横纹肌母细胞.除1例局部区域外,瘤细胞异型性不明显或仅有轻度异型性,核分裂象为1～2个/10 HPF.部分区域内可见炎性或液化性坏死.免疫表型:瘤细胞弥漫强阳性表达CK(AE1/AE3),不同程度表达CD31和FLI1,INI-1标记显示无缺失,Ki-67平均增殖指数约5％.本组6例中2例复发,其中1例在短期内发生双肺转移.结论 PHE是一种局部易复发偶有转移的中间性血管内皮瘤.