血管平滑肌脂肪瘤10例临床病理分析

目的探讨血管平滑肌脂肪瘤(AML)、上皮样血管平滑肌脂肪瘤(EAML)的临床病理学及良、恶性的诊断和鉴别诊断。方法对10例AML及其亚型EAML进行临床病理学及免疫组化观察,随访和复习相关文献。结果经典型AML4例,肌瘤型1例,EAML5例。其中4例发生在肾、肝和子宫。镜检:瘤细胞上皮样、疏松、不规则结节状或片状或围绕血管生长,核圆、稍大,轻度异型性,核分裂象1个/50HPF;发生于阔韧带的1例EAML,瘤细胞上皮样,核大、深染、核异型,可见核内包涵体,核分裂象1个/10HPF,胞质明显嗜酸,肿瘤性坏死和肿瘤组织侵及同侧卵巢及盆腔壁;随访近5年,疑似局部复发和肝转移。其他病例均未见复发转移。免疫组化:HMB45、melanA和SMA(+),desmin部分(+),S-100蛋白、CD34、CK、EMA和CgA(-)。结论 AML大部分为良性病变,但瘤组织出现坏死、瘤细胞核的活性增加、核的不典型性和肾外扩散时应高度考虑为恶变。对EAML病例即使是良性形态也必须密切随访,同时应与相似形态的肿瘤鉴别。     

上皮样血管平滑肌脂肪瘤临床病理观察

目的观察上皮样血管平滑肌脂肪瘤(epithelioid angiom yolipoma,EAML)的病理特征、免疫表型,探讨其组织起源。方法对1例肝脏、2例肾脏EAML的临床表现、病理组织学形态、免疫表型进行观察并复习文献。结果镜下见肿瘤主要由成熟脂肪细胞、上皮样细胞组成,并见薄壁血管及泡沫细胞;上皮样细胞呈散在或片状分布在脂肪组织间,细胞体积大,胞制质透亮,核大居中,呈圆形或卵圆形,无异型及核分裂象,间质内见少量淋巴细胞;免疫组化:上皮样细胞vimentin、S-100、HMB-45阳性,Desmin、SMA灶性阳性,EMA阴性,Ki-67阴性。结论 EAML是一种十分少见且具有恶性潜能的间叶源性肿瘤,易被误诊为恶性肿瘤,免疫组化对其鉴别诊断有重要意义。     

肾脏上皮样血管平滑肌脂肪瘤9例临床病理分析

目的 :探讨肾上皮样血管平滑肌脂肪瘤(angiomyolipoma,AML)的临床病理学特征及诊断和鉴别方法。方法:分析9例原发于肾脏的上皮样AML患者的临床资料、组织形态学、免疫组织化学检测结果及随访情况,并结合相关文献进行讨论。结果:9例患者中男性5例,女性4例,临床表现主要为腰痛、发热,可有血尿、消瘦等伴随症状,均无结节性硬化性综合征及恶性黑素瘤的临床证据。光学显微镜下,肿瘤细胞呈上皮样改变,细胞质透明,核呈圆形或卵圆形,可见小核仁,围绕血管排列,可伴有部分梭形细胞。免疫组织化学检查显示,9例患者的肿瘤组织中HMB45、Melan A、SMA均为阳性;5例Desmin阳性,6例波形蛋白(Vimentin)阳性,3例S-100阳性,6例部分瘤细胞CD34阳性;CK细胞角蛋白(cytokeratin,CK)均为阴性;Ki-67阳性指数为1%~15%(平均8%);1例为会诊病例没有补做TFE-3,只有8例TFE-3的结果,且均为阴性。结论:上皮样AML以瘤细胞呈上皮样改变、围绕血管分布为特点,免疫组织化学检测以黑素细胞标志物最为灵敏。临床应结合患者的临床表现和相关病史,并依据病理学特征及相关免疫组织化学综合判断以确诊。     

肾脏上皮样血管平滑肌脂肪瘤临床病理观察

目的 报道一例肾脏上皮样血管平滑肌脂肪瘤,对其临床病理特点及鉴别诊断进行讨论.方法 对手术切除的肾脏及其肿瘤进行常规病理检查并复习文献.结果 患者女性,56岁.右肾下极上皮样血管平滑肌脂肪瘤,位于肾脏实质,界限清楚,向邻近肾脏实质推挤状生长,无浸润.大小约为12.5 cm×9.5 cm×8.0 cm;组织学上主要由单核或多核的上皮样细胞构成,特别是当肿瘤显示不典型的特征时很容易与有些肿瘤混淆.免疫组化显示瘤组织vimetin、SMA、HH35、HMB45、Melan-A、CD68、CD117均阳性.结论 肾脏上皮样血管平滑肌脂肪瘤是十分少见的肿瘤,临床行为属于潜在恶性,易被误诊为恶性肿瘤,确诊主要依靠组织病理学,并辅以免疫组化标记.     

肝脏上皮样血管平滑肌脂肪瘤的临床病理特点

目的:探讨肝脏上皮样血管平滑肌脂肪瘤(hepatic epitheliod angiomyolipoma,HEAML)的临床病理学特点、免疫表型、诊断和鉴别诊断.方法:对5例 HEAML 进行组织学观察及免疫组织化学检测.结果:5例HEAML均为女性,中位年龄44.2岁,3例位于肝左叶,2例位于肝右叶,肿瘤大小2～12 cm.患者无明显临床症状.镜下见肿瘤主要由上皮样细胞构成,弥漫、梁索状或器官样排列,细胞形态呈不同程度的异型性,胞质丰富,嗜酸或透明状,并可见多核或奇异形核细胞.免疫组化示HMB45、Melan-A、Actin和s-100均有阳性表达.结论:肝脏的上皮样平滑肌脂肪瘤是一种恶性潜能未定的肿瘤,其形态学表现较为多样,应与肝脏的其他肿瘤相鉴别,免疫组化对鉴别具有重要意义.     

肝脏上皮样血管平滑肌脂肪瘤声像图特征

目的 探讨肝脏上皮样血管平滑肌脂肪瘤的常规超声和CEUS特征。方法 回顾性分析16例经病理证实的肝脏上皮样血管平滑肌脂肪瘤患者常规声像图及其中8例患者的CEUS资料。结果 常规超声:16例中,单发14例,多发2例,共18个病灶;其中位于肝右叶9个,肝左叶8个,尾状叶1个;边界清楚14个,边界不清4个;有包膜病灶4个,无包膜病灶14个;低回声病灶13个,高回声病灶2个,囊实混合回声病灶3个;CDFI血流分级0级3个,Ⅰ级1个,Ⅱ级12个,Ⅲ级2个。肿瘤最大径1.5~30.0 cm,中位最大径7.5 cm;背景肝均无肝硬化表现。CEUS:8例共8个病灶中,7个呈"快进慢出"强化模式,1个呈"快进快出"强化模式。动脉期未见明显从周边向中心或从中心向周边增强。结论 肝脏上皮样血管平滑肌脂肪瘤的常规超声及CEUS表现有一定特征。     

肝上皮样血管平滑肌脂肪瘤1例

患者女性,41岁。右肝区阵发性胀痛伴右背部针刺样疼痛不适6个月。查体：肝区略感触痛,叩痛明显。超声检查示肝右后叶有实性强回声光团,大小6．1cm×5．5cm×3．8锄,形态规则,边界清,内部回声欠均匀。实验室检查：AFP（-）。临床诊断：肝右叶脂肪瘤,不除外脂肪肉瘤。行肝内肿块切除术,术中见肝右后叶-5．5cm×5cm×5cm大小肿块,边界清楚,行肿块剥除术,并将肿块送病理检查。     

肝单形性上皮样血管平滑肌脂肪瘤2例临床病理分析

目的 探讨肝单形上皮样血管平滑肌脂肪瘤（MEA）的临床病理学特征及诊断、鉴别诊断要点。方法 对2例MEA进行临床病理学分析、免疫组织化学及电镜研究。结果 患者临床无症状。肿瘤由形态多样的上皮样细胞构成，胞质透亮或嗜酸，无脂肪细胞及异常血管；HMB45（ ），SMA和Vim部分（ ），Des少烤（ ），S-100弱（ ），CK和AFP（-），CD34血管内皮细胞（ ）。电镜下瘤细胞胞质中微丝束上有高电子致密度的凝集物显示，可见黑色素样小体。结论 肝MEA是极为罕见的间叶性肿瘤，组织起源有争议，其诊断及鉴别诊断主要依靠组织病理学、免疫组织化学及电镜检查。     

[1]超声造影鉴别诊断肝上皮样血管平滑肌脂肪瘤和肝癌的临床价值研究

2成都新世纪妇女儿童医院?影像科??610031 摘要 目的 分析超声造影（CEUS）鉴别诊断肝脏上皮样血管平滑肌脂肪瘤（EAML）和肝癌的价值。方法 回顾性分析40例肝脏EAML患者（EAML组）和40例肝癌患者（肝癌组）的CEUS资料。用Qontrast软件对图像进行定性和定量分析,定性分析结果包括不同实时增强时期的强化程度,定量分析结果包括造影剂抵达时间、峰值强度、达峰时间、增强斜率和开始消退时间。结果 常规超声检查显示,与肝癌组比较,EAML组回声程度较低、病灶数量较多（P＜0.05）,两组血流信号强度、病灶位置和病灶边缘特点的比较无统计学差异（P＞0.05）。与肝癌组比较,EAML组向心性增强例数较多,延迟相强化程度较低（P＜0.05）。两组动脉相和门脉相增强程度比较无统计学差异（P＞0.05）。与肝癌组比较,EAML组峰值强度较高,而达峰时间和开始消退时间较短（P＜0.05）。两组增强斜率和抵达时间的比较无统计学差异（P＞0.05）。结论 肝脏EAML和肝癌存在不同的血流灌注特征,CEUS对鉴别诊断这两种疾病有一定价值。     

肾上皮样血管平滑肌脂肪瘤临床病理分析

目的探讨肾上皮样血管平滑肌脂肪瘤(EAML)的临床病理特征、诊断、鉴别诊断及预后。方法对11名肾EAML患者的12例肾进行光镜观察和免疫组化染色。结果 11名患者年龄25～52岁,中位年龄36.2岁。其中5人无症状,5人主诉为腰部酸痛,1人表现为发热。肿瘤直径1.3～17cm,平均9.2cm。术前6例疑为肾癌(RCC)。组织学表现肿瘤以增生的上皮样细胞为特征,胞质丰富嗜酸性,细胞核有异型性,核分裂象可见;可伴有出血、坏死。12例肾EAML中小细胞型6例,大细胞型3例,中间型3例,均伴有经典的肾血管平滑肌脂肪瘤(AML)图像。免疫组化:瘤细胞vimentin、SMA和HMB45(+),AE1/AE3、EMA、CD10、CD34、S-100和CD117均(-),1例Ki-67(+)。术后6名患者得到随访(平均24个月),均健在。结论肾EAML的特征以上皮样细胞增生为主,临床和组织学易与RCC或其他肿瘤相混淆,免疫组化有助于鉴别诊断。瘤细胞不典型增生、核分裂、出血和坏死,甚至肾静脉内瘤栓可能提示肿瘤的恶性潜能,但不能作为诊断恶性的依据,诊断恶性必须要有明确的远处转移。     

Epithelioid angiomyolipoma of the liver: CT and MRI features

The purpose of our study was to retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. We reviewed the CT/MRI findings of six lesions of Epi-HAML and found absence of adipose tissue in the lesions. In addition, recognizing the imaging features of no capsule, and hypervasularity with central punctiform or filiform vessels as a characteristic enhancement may distinguish Epi-HAML from other hepatic tumors.     

Epithelioid angiomyolipoma of the pancreas: A case report and review of the literature

BACKGROUNDAngiomyolipomas (AMLs), belonging to the family of mesenchymal tumors, are considered benign lesions that occur mostly in the kidney or as a part of tuberous sclerosis. Epithelioid AML (EAML) is a rare type of AML that appears to have malignant potential. Extrarenal AMLs usually occur in the liver according to the retrieved literature reports. There have been only two previous reports of monofocal primary AML of the pancreas; however, no cases of primary monotypic EAML of the pancreas have been reported.CASE SUMMARYAn asymptomatic 59-year-old woman incidentally revealed a tumor during abdominal ultrasound examination. Routine blood tests and physical examination were within normal limits. Abdominal ultrasound revealed a 1.9-cm hypoechogenic mass in the tail of the pancreas, clearly visualized by endoscopic ultrasound. However, contrast-enhanced abdominal computed tomography scans did not demonstrate the lesion. A subsequent gadolinium-enhanced magnetic resonance imaging scan showed that the lesion had some characteristic manifestations. The lesion was initially thought to be a neuroendocrine tumor (asymptomatic PanNET). After surgical resection, histopathology and immunohistochemistry confirmed the diagnosis of EAML. At the 6-mo follow-up, no recurrence, spread, or metastasis was identified on computed tomography or magnetic resonance imaging.CONCLUSIONThe preoperative diagnosis of pancreatic AML is extremely difficult. Imaging techniques are essential for providing valuable morphological features for differential diagnosis.     

Hepatic angiomyolipoma

The imaging and pathological appearances of a hepatic angiomyolipoma are presented. On ultrasound (US), the mass was well-defined and echogenic. On computed tomography (CT), the lesion appeared of soft tissue density with peripheral foci of lower density. The case illustrates the difficulty of detecting the fatty content of a tumor by CT when the fatty content represents only a small portion of the lesion. Fine-needle aspiration (FNA) cytology and needle biopsy did not yield diagnostic material, and the tumor was resected.An invited commentary on this article follows on pp. 552–553.     

Renal angiomyolipoma.

An unusual case of a patient with tuberous sclerosis who presented initially with renal symptoms is presented. Electromicroscopic observations showed the smooth muscles to contain myofilaments and an unusual amount of glycogen. Although the response of the tumor vessels to epinephrine in angiomyolipoma is considered similar to that of renal cell carcinoma in that no vasoconstriction occurs, the reverse occurred in our case. A thin, lucent cleavage line around portions of the periphery of the angiomyolipoma was also noticed. Isolated angiomyolipoma in patients without tuberous sclerosis is difficult to differentiate from renal cell carcinoma; therefore, radical nephrectomy is usually the treatment of choice. Angiomyolipomas associated with tuberous sclerosis are easier to diagnose and are multiple and bilateral; therefore, a more conservative approach usually is indicated.     

儿童上皮样血管内皮瘤1例报道

上皮样血管内皮瘤(epithelioid heman-gioendothelioma,EHE)是近20年才认识的一种较少见的低度恶性血管源性肿瘤,在病理学特征、生物学行为和预后方面都介于血管瘤和血管肉瘤之间,全身各部位均可发生,以软组织多见.我院收治1例发生于儿童肠道的上皮样血管内皮瘤,现结合文献对其诊断、鉴别诊断和治疗进行探讨.