血管周上皮样细胞肿瘤

血管周上皮样细胞（PECs）起源于间叶细胞,分布于毛细血管周围,是一种多潜能细胞,其发生的肿瘤称为血管周上皮样细胞肿瘤（PEComas）。PEComas是近10几年来新提出的一组间叶源性肿瘤,包括血管平滑肌脂肪瘤、肺的透明细胞“糖”瘤、淋巴管平滑肌瘤病、镰状韧带的透明细胞肌黑色素细胞肿瘤及其它部位罕见的透明细胞瘤。目前尚无定义良、恶性的诊断标准。部分PEComas与结节性硬化综合征的遗传学相关。     

肝血管平滑肌脂肪瘤1例报道

血管平滑肌脂肪瘤(Angiomyolipoma,AML)为少见病,多见于肾脏,发生在肝脏者罕见且成分复杂,术前诊断困难,因此,误诊率较高.作者通过1例较典型的肝血管平滑肌脂肪瘤完整病例的介绍及相关文献复习,以期提高对肝血管平滑肌脂肪瘤的认识及诊断水平.     

肝上皮样血管内皮瘤1例

上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)是一种病因及发病机制不明,累及单个或多个器官的血管源性肿瘤,具有低至中度恶性潜能,呈慢性进行性发展过程.肝脏是EHE受累的常见器官之一.Ishak等[1]在1984年首次报道了肝上皮样血管内皮瘤(hepatic epithelioid hemangioendothelioma,HEHE),此后相关报道逐渐增多.影像学检查是诊断HEHE常用的手段之一,如螺旋X线计算机断层摄影术(CT)、磁共振成像(MRI)、核医学检查等,但存在一定的局限性.我们在此报道1例病史长达17年的HEHE患者的肝脏CT灌注特点及其在HEHE诊断中的价值.     

肺恶性血管周上皮样细胞肿瘤合并肺腺癌首例报道

目的:分析肺恶性血管周上皮样细胞肿瘤（PEComa）合并腺癌的发病机制、临床表现、诊断、治疗及预后。方法:分析2020年8月南方医科大学附属东莞人民医院病理科确诊的1例肺原发恶性PEComa合并腺癌病例,整理患者的临床资料、病理诊断要点、治疗方案及预后等,并进行文献复习。首先以“恶性血管周上皮样细胞肿瘤”+“肺”+“腺...     

肝脏上皮样血管平滑肌脂肪瘤CT及MRI影像学与病理特征探讨

目的 探讨肝脏上皮样血管平滑肌脂肪瘤(HEAML)的影像学与病理特征.方法 选择四川省肿瘤医院2012-09～2019-09经手术病理证实的15例HEAML患者的临床资料,总结其影像学与病理特征.结果 15例HEAML平扫均呈稍低密度/信号,边界清楚,5例病灶内见少许脂肪成分.增强扫描动脉期15例均呈明显不均匀强化,其...     

4例肝脏血管周上皮样细胞瘤临床病理特点分析

正血管周上皮样细胞(perivascular epithelioid cell,PEC)形态特征为细胞呈上皮样,胞质透亮或淡嗜伊红色,PAS染色呈阳性,核小,圆形或卵圆形,可有小核仁。PEC常围绕血管呈放射状排列。邻近血管的PEC呈上皮样,而远离血管的PEC呈梭形,似平滑肌细胞;上皮样细胞和梭形细胞的相对比例有较大差异。     

肝血管平滑肌脂肪瘤的影像学征象

肝血管平滑肌脂肪瘤(HAML)顾名思义主要由血管、平滑肌和脂肪3种成分组成,典型的结构为分化不同阶段的平滑肌样细胞与脂肪细胞的相混杂,点缀着畸形的血管.肾脏血管平滑肌脂肪瘤多见,发生于肝脏者极少.     

肝上皮样血管内皮细胞瘤3例报告

<正>肝上皮样血管内皮细胞瘤(hepatic epithelioid hemangioendothelioma, HEHE)是一种较为少见的血管源性肿瘤,生物学行为具有恶性转移潜能[1]。HEHE起病隐匿,缺少特异性临床表现及实验室检查,易出现漏诊误诊。回顾性分析收治的3例HEHE患者临床资料,并结合文献探讨HEHE的诊断及治疗,为临床医师作出合理诊疗决策提供参考。1 病例资料病例1:患者男性,58岁,     

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。     

胃淋巴上皮瘤样癌表现为SMTs的病例分析及解读

内镜微创技术的不断进步与发展,使得更多黏膜下肿物（SMTs）逐渐被发现、诊断及治疗。胃肠道SMTs最常见的是平滑肌瘤、间质瘤、脂肪瘤、类癌,其他如异位胰腺、神经鞘瘤、错构瘤及血管球瘤等则较为少见。2016年5月本中心诊断并治疗1例以SMTs的形式表现且病理诊断为胃淋巴上皮瘤样癌（Lymphoepithelioma-like gastriccarcinoma,LELC）的患者,该病最早由Watanabe等[[[] Watanabe H,Enjojim,Imai T.Gastric carcinoma with lymphoid stroma.Its morphologic characteristics and prognostic correlations[J].Cancer,1976,38(1):232-243.]]于1976年报道,目前国内外报道例数极少,缺乏综合临床资料,术前诊断较困难。本文结合相关文献分析及病理学、免疫学特点,报道如下。     

肝脏上皮样血管平滑肌脂肪瘤实时超声造影征象研究

目的 分析总结肝脏上皮样血管平滑肌脂肪瘤(EAML)的灰阶超声、多普勒超声(CDFI)和超声造影(CEUS)表现。方法 回顾性分析我院经手术后组织病理学检查证实的20个EAML患者的临床、灰阶超声、CDFI和CEUS资料。结果 20例病灶均为单发,最大径为(49.23±10.3)mm;病灶内部回声为低回声14例(70.0%),高回声3例(15.0%),高低混合不均匀回声3例(15.0%);CDFI显示0级4例(20.0%),I级3例(15.0%),II级10例(50.0%),III级3例(15.0%);阻力指数(RI)为0.46±0.34;CEUS显示所有病灶动脉期都表现为快速高增强,其中13例(86.6%)为整体均匀增强,门静脉期高增强12例(80.0%),等增强2例(13.3%),低增强1例(6.6%),延迟期仍表现为高增强10例(66.6%),等增强4例(26.6%),低增强1例(6.6%)。结论 EAML灰阶超声主要表现为低回声,CDFI显示为富血供、低阻力型血流,CEUS主要表现为快进慢退/不退,三者结合或有助于提高诊断的准确性。     

Improving recognition of hepatic perivascular epithelioid cell tumor:Case report and literature review

A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.     

Chronic hepatitis C is a common associated with hepatic granulomas

AIM： To determine the most frequent etiologies of hepatic epithelioid granulomas, and whether there was an association with chronic hepatitis C virus （HCV）. METHODS： Both a retrospective review of the pathol- ogy database of liver biopsies at our institution from 1996 through 2006 as well as data from a prospective study of hepatic fibrosis markers and liver biopsies from 2003 to 2006 were reviewed to identify cases of hepatic epithelioid granulomas. Appropriate charts, liver biopsy slides, and laboratory data were reviewed to determine all possible associations. The diagnosis of HCV was based on a positive HCV RNA. RESULTS： There were 4578 liver biopsies and 36 （0.79%） had at least one epithelioid granuloma. HCV was the most common association. Fourteen patients had HCV, and in nine, there were no concurrent condi- tions known to be associated with hepatic granulomas. Prior interferon therapy and crystalloid substances from illicit intravenous injections did not account for the finding. There were hepatic epithelioid granulomas in 3 of 241 patients （1.24%） with known chronic HCV enrolled in the prospective study of hepatic fibrosis markers. CONCLUSION： Although uncommon, hepatic granu- Iomas may be part of the histological spectrum of chronic HCV. When epithelioid granulomas are found on the liver biopsy of someone with HCV, other clini- cally appropriate studies should be done, but if nothing else is found, the clinician can be comfortable with an HCV association.     

胃肠道间质瘤32例临床病理分析及免疫组化研究

目的：探讨胃肠道间质瘤的临床病理及免疫组化特点和诊断标准。方法：对32例胃肠道间质瘤进行常规病理检查及免疫组化染色。结果：胃肠道间质瘤由梭形细胞和上皮样细胞组成，大多数病例CD117和CD34标记阳性。结论：胃肠道间质瘤是胃肠道常见的非上皮性肿瘤，缺乏定向分化。CD117、CD34标记阳性对胃肠道间质瘤的诊断具有重要的价值。     

胰腺血管周上皮样细胞肿瘤的临床病理特点

目的:探讨胰腺血管周上皮样细胞肿瘤(perivascular epithelioid cell tumors,PEComas)的临床病理学特征、诊断、鉴别诊断、治疗方法和预后.方法:对1例罕见的胰腺PEComas进行光镜观察、免疫组织化学染色,并结合文献探讨该病的临床病理学特点.结果:肿瘤位于胰头部,光镜下可见肿瘤内含...     

Clinicopathologic study of primary intestinal B cell malignant lymphoma

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Visceral bacillary epithelioid angiomatosis: possible manifestations of disseminated cat scratch disease in the immunocompromised host: a report of two cases

Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.     

Primary pleural epithelioid hemangioendothelioma (EHE)--two cases and review of the literature

Introduction: We present two cases with symptoms of progressively worsening cough, dyspnea, decreased exercise tolerance and right‐sided back pain in the first case and upper respiratory symptoms characterized by cough and a low grade fever in the second case. Methods: Report of two cases. Results: The initial chest X‐ray in both the cases showed pleural effusion. Further imaging with computed tomography of the chest confirmed the effusion in both cases. Thoracentesis was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary pleural epithelioid hemangioendothelioma was made. Conclusions: Both the cases had progressive clinical deterioration despite chemotherapy with Taxol and Bevacizumab in one case and carboplatin, etoposide, and bevacizumab, in the second case. Both developed metastatic disease to lungs and died. Please cite this paper as: Lazarus A, Fuhrer G, Malekiani C, McKay S and Thurber J. Primary pleural epithelioid hemangioendothelioma (EHE) – two cases and review of the literature. Clin Respir J 2011; 5: e1–e5.     

Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.RESULTS: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.CONCLUSION: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.