子宫恶性血管周上皮样细胞肿瘤2例报道

正血管周上皮样细胞肿瘤(perivascular epithelioid cells tumor,PEComa)是一组在形态学及免疫表型上显示血管周上皮样细胞特征的间叶性肿瘤,包括肾血管平滑肌脂肪瘤(angiomyolipoma,AML)、肺透明细胞糖瘤(clear cell "sugar"tumor,CCST)、淋巴管肌瘤和淋巴管肌瘤病(lymphangioleiomyoma and lymphangiomyomatosis,LAM and LAMs),     

PEComa研究进展

子宫血管周上皮细胞恶性肿瘤(PEComa)是一组较少见组织学和免疫表型上具有血管周上皮样细胞特征的间叶肿瘤,瘤细胞胞质透明或淡嗜伊红颗粒状,免疫组化检测表达黑色素瘤抗体HMB45、Melan-A(A103)及肌源性抗体SMA.包括肝肾血管平滑肌脂肪瘤(angiomylipoma,AML),肺的透明细咆"糖"瘤(Clear cell"sugar"tumor of the lung,CCST),淋巴管肌瘤病(Lymphangiomyoleiomymatosis,LAM),镰状韧带、圆韧带的透明细胞肌黑色素瘤(clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres,CCMMT)以及胰腺、直肠、腹腔浆膜、子宫、外阴、大腿和心脏等特殊部位的透明细胞肿瘤.现就该瘤近年研究进展做一综述.     

子宫血管周上皮样细胞肿瘤一例报道及文献复习

目的探讨子宫血管周上皮样细胞肿瘤(PEComa)的临床病理特点、诊断及鉴别诊断。方法对1例子宫PEComa患者资料进行分析并复习相关文献。结果本例子宫PEComa位于肌壁间,瘤细胞呈上皮样或梭形,围绕血管呈巢状排列,胞浆透明或嗜酸,免疫组化染色HMB45、SMA阳性,随访15个月无瘤生存。结论发生于子宫的PEComa是一种少见的间叶性肿瘤,术前检查容易误诊,病理组织学及免疫组化特征性抗体HMB45、SMA表达有助于确诊。此瘤多数为良性,亦有恶性子宫PEComa的报道,其生物学行为尚不确定,有待进一步积累病例进行分析,为临床病理医师提供诊断、随访依据。     

肝血管平滑肌脂肪瘤3例临床病理分析

目的对肝血管平滑肌脂肪瘤的临床病理特点、免疫组化、诊断及鉴别诊断进行探讨.方法3例肝AML标本均经常规处理,HE染色和S-P法免疫组化染色,光镜观察.结果肿瘤位于肝左叶2例,肝右叶1例.3例肿瘤内均可见平滑肌、脂肪、畸形血管及造血细胞,其中上皮样平滑肌细胞为肿瘤的主要成分(＞90%),瘤细胞排列呈弥漫片状、条索状或小梁状.1例肿瘤中可见炎性假瘤样区域.免疫组化染色上皮样平滑肌细胞表达HMB45强阳性.术后随访3～31个月,未见肿瘤复发.结论肝AML的形态学特点有别于肾AML,肿瘤中3种成分的比例、瘤细胞形态及组织结构均有很大变化,必须与多种肿瘤相鉴别.平滑肌细胞表达HMB45强阳性,是诊断肝AML的可靠依据.     

子宫恶性血管周上皮样细胞肿瘤3例临床病理观察

目的探讨子宫恶性血管周上皮样细胞肿瘤(PEComa)的临床病理特点、免疫组化、诊断与鉴别诊断、治疗及其预后。方法对3例子宫恶性PEComa的临床资料、组织形态学、免疫组化及预后进行探讨,并复习相关文献。结果子宫恶性PEComa是一组共同表达黑色素和肌源性标记为特征的肿瘤,肿物均未见明显的包膜,由梭形细胞或上皮样细胞构成,呈片状、巢状排列,细胞具有异型性,核分裂象可见,出血、坏死明显。免疫组化:HMB45(3/3)、melan A(1/3)、SMA(2/3)、desmin(2/3)、NSE(1/3)和CD10(2/3)(+),Ki-67阳性率为3%~30%。结论子宫恶性PEComa少见,容易误诊为平滑肌肉瘤或透明细胞癌等,临床以手术切除为主,放、化疗意义不明确。     

肝血管周上皮样细胞肿瘤临床病理观察

目的探讨血管周上皮样细胞肿瘤(PEComa)的临床病理特点及鉴别诊断。方法报道1例发生于肝的血管周上皮样细胞肿瘤并对其临床表现、组织学形态、免疫表型及生物学行为进行分析,并复习相关文献。结果血管周上皮样细胞肿瘤是起源于血管周细胞的间叶性肿瘤,组织学上由单一的上皮样细胞组成,不含厚壁血管及脂肪组织,该肿瘤黑色素标记物HMB45(+)。结论血管周上皮样细胞肿瘤是一种非常罕见的间叶组织来源的肿瘤,具有独特的形态学和免疫组化特征,生物学行为不确定。临床以手术切除为主,且建议术后长期随访。     

肾脏上皮样血管平滑肌脂肪瘤1例报道并文献复习

<正>血管平滑肌脂肪瘤(angiomyolipoma,AML)是间充质组织来源的常见良性肿瘤,分经典型和上皮样。经典型肾血管平滑肌脂肪瘤组成成分上包括不同比例的厚壁血管、脂肪组织和平滑肌组织[1]。肾上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma of kidney,REAML)是一种罕见的肾间质肿瘤,属于血管周围上皮样细胞肿瘤家族的一员,主要由排列成片的大量增生性上皮样细胞组成,罕有脂肪成分[2,3],具有恶性潜能。现将本院收治的1例肾脏上皮样血管平滑肌脂肪瘤诊疗情况报道如下。     

肺上皮样血管周细胞瘤1例临床病理观察及文献复习

目的观察肺上皮样血管周细胞瘤(neoplasms with perivascular epithelioid cell,PEComa)的临床病理特点。方法对本院2008年会诊的1例肺PEComa进行临床病理资料分析和光镜、免疫组化标记观察并复习相关文献。结果肺PEComa临床表现与其他肺肿瘤比较无独特性。其是起源于血管周细胞的良性间叶性肿瘤,镜检瘤细胞呈圆形、卵圆形,富含糖原,细胞核无明显异型,未见核分裂像及瘤性坏死。免疫组化标记该肿瘤细胞特异性表达黑色素标记HMB-45及S-100。结论 PEComa发生于肺比较罕见,与肺的其他恶性肿瘤鉴别需借助病理及免疫组化标记。     

肝脏血管平滑肌脂肪瘤临床病理特点分析

目的探讨肝脏血管平滑肌脂肪瘤(hepatic angiomylipoma,HAML)的临床病理特点、诊断及鉴别诊断。方法对3例HAML进行临床病理学观察及免疫组化标记并复习有关文献。结果 3例HAML,年龄分别为46岁,38岁和16岁,均无结节硬化症。术前影像诊断为肝占位。眼观:肿瘤直径7~20cm,肿瘤境界清楚,无包膜,质地脆、易碎,可见出血、坏死。镜检:瘤组织由上皮样细胞、平滑肌样细胞、脂肪组织和厚壁血管组成。上皮样细胞体积大或巨大,呈多边形,胞质丰富,嗜酸性或空泡状,核仁明显。平滑肌样细胞呈梭形,瘤细胞弥漫性或巢团状排列。免疫组化标记:3例病例标本均表达Melan-A,例二和例三表达HMB45,例一和例二表达S-100。SMA和CD34三例病例均表达。Ki-67例一约10%(+),例二和例三均5%(+)。结论HAML是一种罕见的间叶组织源性肿瘤,组织学上由上皮样细胞、平滑肌样细胞、脂肪组织和厚壁血管组成,瘤细胞较为特异的表达Melan-A、HMB45及S-100。免疫组化标记可与其它肝脏肿瘤鉴别。     

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。     

肝镰状韧带透明细胞肌黑色素瘤临床病理观察

目的探讨透明细胞肌黑色素瘤（CCMMT）的临床表现、病理特征及鉴别诊断。方法对1例肝镰状韧带CCMMT进行病理组织学、免疫组化观察，并复习相关文献。结果CCMMT是软组织良性肿瘤，好发于女性，常为无痛性肿块，以镰状韧带为好发部位，组织学以上皮样透明细胞及平滑肌样梭形细胞为主，伴同步增生丰富的毛细血管，免疫组化HMB45、SMA和S-100（＋）；结论血管周上皮样细胞肿瘤PEComa是WHO确立的软组织肿瘤新类型，肝镰状韧带CCMMT是这个肿瘤家族的代表性成员之一，具有显著的部位特点，组织学以透明细胞、梭形平滑肌样细胞分化为主，特异性免疫标记有助诊断。该肿瘤应与软组织透明细胞肉瘤、恶性透明细胞间皮瘤等进行鉴别。     

Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features—analysis of 7 cases and review of the literature

Objective

To evaluate the dynamic CT, MRI, ultrasonography, and pathologic features of hepatic perivascular epithelioid cell tumor (PEComa), improving the understanding and diagnosis of the tumor.

Methods

A retrospective analysis of CT, MRI, ultrasonography, and pathologic features of 7 hepatic PEComas diagnosed by pathology during 1st January 2005 to 1st September 2011 in our hospital.

Results

The performance of dynamic CT, MRI, and ultrasonography revealed that lesions were regular masses with well-defined borders, the maximum diameters were 2.5–8.5?cm (mean?=?4?cm), density was homogeneous, contrast-enhanced CT and MRI showed the lesions were significantly and heterogeneously enhanced on arterial phase, less enhanced on portal venous phase, and slightly hypodense on delayed phase. One patient had multiple hepatic lesions and had delayed enhancement. There were no backgrounds of hepatitis and cirrhosis, enlarged lymph nodes, or distant metastases. Pathology showed the gross appearance of the tumor was smooth. Tumor cells were round or polygonal, with clear boundaries and clear membranes, and had abundant translucent cytoplasm. Nuclei were round, with medium size. Tumor cells were epithelial-like cells and arranged in dense sheets. Immunohistochemistry showed that most of them were positive in HMB45 and MelanA, S-100, SMA, while negative in CgA, Syn, CK, CD117, CD10, and CD34.

Conclusions

Dynamic CT, MRI, ultrasonography, and pathology of PEComa had some characteristics of benign tumor’s performance. Enhanced scan showed PEComa quickly enhanced on arterial phase and enhanced less on portal venous phase. Knowing these characteristics could help to improve the understanding and diagnosis of hepatic PEComa.     

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。     

TTF-1在肺癌诊断及鉴别诊断中的应用价值

目的 评价甲状腺转录因子-1（TTF-1）在原发性肺癌中的表达特异性及敏感性，探讨TTF-1对肺癌诊断和鉴别诊断的潜在价值。方法 原发性肺癌95例，其中腺癌52例，肺鳞癌39例，细支气管肺泡癌1例，肺小细胞癌3例；正常肺组织4例，上皮样胸膜间皮瘤4例及186例人体其他部位的正常组织和肿瘤组织做成组织芯片；采用EnVision方法进行TTF-1检测。结果 TTF-1阳性信号的定位在细胞核。44／52例（84．6％）原发性肺腺癌、2／39例（5％）肺鳞癌、1／1例肺小细胞癌和2／3例细支气管肺泡癌表达TTF-1，4例上皮样胸膜间皮瘤均不表达TTF-1，其他部位的正常组织和肿瘤组织（除1例甲状腺癌外）均不表达TTF-1。另外，6／7例肝细胞出现很强的胞质（＋），胆管上皮（-）。结论 TTF-1在原发性肺腺癌中的表达具有较高敏感性和特异性，提示TTF-1可作为一种有价值的标记用于原发和转移性肺腺癌的鉴别；它对于肺原发性低分化腺癌和鳞癌的鉴别也有一定价值，还具有鉴别肝细胞癌和胆管癌的潜在价值。     

Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review

Perivascular epithelioid cell tumour (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, with few reports of paediatric cases, and there is no standardized treatment for the tumour type. Here, the case of a 17-year-old female, who presented with painless haematochezia for 2 days and was diagnosed with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis after serial examination, is presented. She was treated by surgical resection of the tumour and cytotoxic chemotherapy comprising 900 mg/m² gemcitabine and 100 mg/m² docetaxel every 3 weeks for six cycles. Haematochezia did not recur, and complete response was achieved, with progression-free survival at the 24-month follow-up examination. Surgical resection with adjuvant conventional cytotoxic chemotherapy may be considered as an option for treating gastrointestinal PEComa.     

Rare case of angiomyolipoma of the larynx

We report a rare case of angiomyolipoma (AML) of the larynx. AML belongs to the family of perivascular epithelioid cell tumors (PEComas). We review the literature on PEComas and describe differences in immunohistochemical findings between renal AML and AML in the head and neck region.     

Hepatic epithelioid hemangioendothelioma: Update on diagnosis and therapy

With an estimated incidence of only 1-2 cases in every 1 million people, hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types: solitary, multiple, and diffuse. Both the etiology and characteristic clinical manifestations of HEHE are unclear. However, HEHE has a characteristic appearance on imaging including ultrasound, magnetic resonance imaging, and positron emission tomography/computerized tomography. Still, its diagnosis depends mainly on pathological findings, with immunohistochemical detection of endothelial markers cluster of differentiation 31 (CD31), CD34, CD10, vimentin, and factor VIII antigen as the basis of diagnosis. Hepatectomy and/or liver transplantation are the first choice for treatment, but various chemotherapeutic drugs are reportedly effective, providing a promising treatment option. In this review, we summarize the literature related to the diagnosis and treatment of HEHE, which provides future perspectives for the clinical management of HEHE.     

CT诊断肾少脂肪血管平滑肌脂肪瘤与透明细胞癌的临床分析

目的研究CT诊断肾少脂肪血管平滑肌脂肪瘤(AML)与透明细胞癌(CRCC)的临床特征,提高诊断的准确率。方法选择AML与CRCC患者各30例为研究对象,分为AML组和CRCC组。回顾性两组患者的CT检查情况以及相关特征指标。结果两组在肿瘤密度、强化程度方面无显著性差异,强化类型上AML组均匀强化显著多于CRCC组,差异有统计学意义(P0.05)。且AML组患者在病灶突出比、皮质掀起征两个方面均大于CRCC组,差异有统计学意义(P0.05)。结论 AML组与CRCC组在CT中表现出的差异较大,AML无论在肿瘤均匀性、病灶突出比还是皮质掀起证都明显大于CRCC,因此应用CT能够准确地鉴别二者。     

肺上皮样血管肉瘤临床病理观察

目的 探讨肺上皮样血管肉瘤（EA）的病理形态学特点及其鉴别诊断。方法 对1例肺EA的组织病理学、免疫组化结果进行观察分析，结合文献进行讨论。结果 肿瘤有大片的坏死出血区。瘤细胞具上皮细胞的形态学特征，异型性明显，CD31和vimentin（＋）。结论 肺EA具有软组织EA的一般病理形态学特征，但出血、坏死更为显著。CD31是敏感性较高的标记物。主要与上皮样血管内皮瘤、恶性间皮瘤、硬化性血管瘤、肺腺癌相鉴别。     

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.