子宫血管周上皮样细胞肿瘤3例临床病理分析

目的探讨子宫血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor, PEComa)的临床病理特征、诊断及鉴别诊断等。方法采用免疫组化EnVision两步法对3例子宫PEComa进行检测,并复习相关文献。结果 3例肿瘤由梭形细胞和上皮样细胞构成,胞质透明至嗜酸性,间质血管丰富,其中1例肿瘤细胞异型性显著,并见出血、坏死。免疫表型:3例HMB-45阳性,2例SMA、Caldesmon阳性,Melan-A、TFE-3、desmin、CD10、CD117和S-100蛋白均阴性,Ki-67增殖指数5%～30%。随访4～55个月,患者均存活。结论子宫PEComa是一种少见的间叶源性肿瘤,结合组织学形态及免疫表型可辅助诊断。     

恶性血管周上皮样细胞肿瘤4例临床病理分析

目的探讨恶性血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的临床病理特点、诊断及鉴别诊断。方法采用免疫组化法及RT-PCR技术对4例恶性PEComa进行检测,并复习相关文献。结果4例中女性3例,男性1例,年龄32~65岁(平均50岁)。肿瘤部位:纵隔1例,阔韧带1例,回肠1例,结肠1例。肿瘤最大径6~18 cm(平均10.75 cm)。镜下见肿瘤细胞大部分为上皮样,细胞异型性大,核仁明显,胞质丰富、空泡状、嗜酸性;肿瘤细胞呈放射状排列在血管周围,血管壁玻璃样变性,可见多核瘤巨细胞,核分裂象多少不等。免疫表型:肿瘤细胞HMB-45(4/4)、Melan-A(3/4)、Cathepsin K(4/4)、α-SMA(4/4)和H-Caldesmon(3/4)阳性;desmin(2/4)和CKpan(1/4)部分阳性;Ki-67增殖指数1%~30%。分子病理检测:例1和例4均未检测到C-Kit及PDGFR-α基因突变信号。结论恶性PEComa诊断依赖于临床特点、病理学形态及免疫表型,治疗以手术完整切除为主,预后好。     

子宫血管周上皮样细胞肿瘤1例

患者,68岁,以"阴道不规则出血3 个月"为主诉,月经规律4～5 天/30天,56 岁绝经.3 个月前阴道出血,量少;3天前阴道出血增多,因症状持续存在而就诊.彩超示:子宫后位,长径38 mm,前后径28 mm,横径35 mm,内膜厚3 mm,轮廓清晰,形态正常,肌层回声不均.子宫宫底部可见大小21 mm ×15 mm...     

女性生殖道血管周上皮样细胞肿瘤临床病理分析

目的 探讨女性生殖道血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor, PEComa)的临床病理特征、诊断与鉴别诊断。方法 收集4例女性生殖道PEComa患者的临床资料,观察其组织形态学特点,采用免疫组化EnVision两步法染色,应用FISH检测TFE3基因重排,分析其临床病理特征并复习相关文献。结果 4例患者均不伴结节性硬化综合征,病变分别位于子宫颈、宫体和阴道残端。肿瘤主要由上皮样细胞排列呈实性片、巢和束状,瘤细胞胞质丰富、透明或嗜酸性、颗粒状,细胞核异型性轻微或显著,核分裂象多少不等。部分病例可见肿瘤性坏死、多核瘤巨细胞和横纹肌样细胞等。免疫表型：3例手术标本中HMB-45、Melan A、MiTF、TFE3、desmin、SMA和h-caldesmon均阳性,S-100和SOX10均阴性。1例会诊病例的HMB-45、MelanA、desmin和SMA均阳性,S-100阴性。3例FISH检测TFE3基因无断裂重排。结论 女性生殖道PEComa易误诊,结合其典型的组织学、免疫表型和分子遗传学特征,有助于提高诊断准确性。     

鼻腔血管周上皮样细胞肿瘤临床病理分析

目的 探讨血管周上皮样细胞分化肿瘤(neoplasm with perivascular epithelioid cell differentiation,PEComa)的临床病理特征,并对国内外相关文献进行复习,以提高对PEComa的认识和病理诊断水平.方法 对1例PEComa进行临床表现分析,对其病理组织学和免疫组化染色进行观察,并结合国内外相关文献复习PEComa的特点.结果 患者发现左鼻腔无痛渐大性肿物伴反复出血1年余.组织病理学形态表现为裂隙状血管周围见片巢状排列的上皮样细胞,细胞胞质透亮,内含淡红色细颗粒状,核小且一致,圆形或卵圆形,核仁小,核分裂罕见.免疫表型:HMB-45、Melan-A、vimentin和SMA(+);S-100蛋白、CK和EMA(-).结论 发生于鼻腔的PEComa罕见,诊断主要依靠病理学,手术切除为首选治疗,其为低度恶性潜能肿瘤,需长期随访.     

肺良性血管周上皮样细胞肿瘤9例临床病理观察

目的 探讨肺良性血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的临床病理特征、诊断及鉴别诊断.方法 回顾性分析9例肺PEComa的临床及影像学资料,总结其组织病理学形态、免疫组化特征及患者预后,并复习相关文献.结果 9例肺PEComa中男性5例,女性4例,4...     

子宫血管周上皮样细胞肿瘤2例报道及文献复习

子宫血管周上皮样细胞肿瘤（perivascular epithelioid cell tumor,PEComa）是一种少见的肿瘤,有其特有的镜下特点和免疫组化特征。现收集2例子宫PEComa病例,并结合文献探讨其诊断和鉴别诊断,以提高对该病的认识。     

4例胃肠道血管周上皮样细胞肿瘤临床病理学分析

目的 探讨胃肠道血管周上皮样细胞肿瘤(perivasculaRepithelioid cell tumor,PEComa)的临床及病理学特点.方法 回顾性复习4例胃肠道原发血管周上皮样细胞肿瘤的病理切片及临床资料,选取典型蜡块做相关的免疫组化染色,抗体包括黑色素相关抗原HMB45、melan-A、肌源性标记抗原SMA、desmin,以及vimentin、CgA、CK、S-100、CD117、CD34.结果 4例PEComas中男性3例,女性1例,年龄分别为36、38、42及45岁.其中2例位于升结肠,1例位于降结肠,1例位于乙状结肠.肿瘤大小4.5～10 cm,境界清楚,切面灰白色,质地均匀,局部可见出血.镜检:肿瘤细胞呈上皮样排列,细胞质丰富,透亮或嗜酸性颗粒状,细胞核空泡状,有明显的核仁,间质富于毛细血管、血窦以及厚壁血管.细胞异型性小,个别病例局部可见轻～中度异型性,分裂象0～3个/10 HPF.免疫组化结果 :肿瘤弥漫表达HMB45(4/4),弥漫或片状表达vimentin(4/4)、SMA(4/4)以及desmin(3/4).CgA、Syn、CK、S-100、CD117、CD10及CD34均阴性.4例患者行局部肠管及肿瘤切除,术后随访8、15、32及36个月均无复发和肿瘤转移.结论 胃肠道PEComa少见,为低度恶性潜能肿瘤,形态类似于软组织和其他部位的同类肿瘤,手术切除为首选治疗.     

子宫血管周上皮样细胞肿瘤临床病理学观察

子宫血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)是一种非常罕见的肿瘤,极易误诊.作者在病理诊断中诊断1例,结合文献复习报道如下.     

硬化性血管周上皮样细胞肿瘤

具有血管周上皮样细胞分化的肿瘤（PEComas）是间胚叶肿瘤的一个家族，包括血管肌脂肪瘤、肺透明细胞“糖”瘤、淋巴管肌瘤病和发生在软组织、内脏器官和皮肤的一组罕见病变。作者描述了一种特殊变异的PEComa，表现为广泛的间质透明变性，该特征先前在这些肿瘤中尚未描述过。     

Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms

Angiomyolipoma (AML) belong to a family of tumors known as perivascular epithelioid cell tumors (PEComas) that share a common immunophenotypic profile of muscle and melanocytic differentiation. These tumors are clonal in nature and have a strong association with tuberous sclerosis. Genetic analyses have reported allelic imbalance at the TSC2 locus on 16p13. In the context of non-tuberous sclerosis complex (TSC), non-lymphangioleiomyomatosis-associated AMLs, and non-renal PEComas, the functional status of the TSC2 signaling pathway has not been reported. Studies over the last several years have uncovered a critical role of the TSC1/2 genes in negatively regulating the Rheb/mTOR/p70S6K cascade. Here, we examined the activity of this pathway in sporadic AMLs and PEComas using immunohistochemical and biochemical analyses. We found increased levels of phospho-p70S6K, a marker of mTOR activity, in 15 of 15 non-TSC AMLs. This was accompanied by reduced phospho-AKT expression, a pattern that is consistent with the disruption of TSC1/2 function. Western blot analysis confirmed mTOR activation concurrent with the loss of TSC2 and not TSC1 in sporadic AMLs. Similarly, elevated phospho-p70S6K and reduced phospho-AKT expression was detected in 14 of 15 cases of extrarenal PEComas. These observations provide the first functional evidence that mTOR activation is common to sporadic, non-TSC-related AMLs and PEComas. This suggests the possibility that mTOR inhibitors such as rapamycin may be therapeutic for this class of disease.     

非特殊性血管周上皮样细胞肿瘤31例的病理学观察

目的 探讨非特殊性血管周上皮样细胞肿瘤(PEComa-NOS)的临床病理学特征,评价恶性血管周上皮样细胞肿瘤(PEComa)的诊断标准.方法 回顾性复习31例PEComa-NOS的临床表现、影像学资料、光镜形态和免疫学表型,分析预后资料.2例为空芯针穿刺活检标本,2例为剖腹探查活检标本,其余27例为手术切除标本.结果 ...     

Atypical epithelioid cell myofibroblastoma of the breast with multinodular growth pattern: A potential pitfall of malignancy

Myofibroblastoma (MFB) of the breast is the prototypical benign spindle cell tumor arising from the mammary stroma. Over the last two decades, several morphological variants of this tumor have been recognized. Epithelioid cell MFB is composed predominantly of neoplastic elements with epithelioid morphology. It represents a potential diagnostic pitfall of malignancy, especially when evaluating small biopsies. We report a unique case of a mammary epithelioid cell MFB composed of large mono- to multi-nucleated cells showing mild to moderate nuclear pleomorphism, predominantly arranged in a multinodular growth pattern. This tumor needs to be distinguished from invasive apocrine, oncocytic, pleomorphic lobular carcinoma, as well as metastases. Immunohistochemistry revealed the fibroblastic/myofibroblatic (positivity for vimentin, desmin, CD34 and focally for α-smooth muscle actin) nature of proliferating cells, and therefore was crucial for a correct diagnosis.     

血管周上皮样细胞和血管周上皮样细胞分化的肿瘤

一、血管周上皮样细胞的由来1991年意大利病理学者Pea等[1]在肺透明细胞糖瘤(CCST)和肾血管平滑肌脂肪瘤(AML)中同时观察到一种胞质透亮至淡嗜伊红色的上皮样细胞.随后的一些研究显示,CCST和AML均表达HMB45,电镜检测也观察到胞质内含前黑色素颗粒.鉴于此,Bonetti等[2]于1992年首次提出血管周上皮样细胞(PEC)这一概念,并在随后的文章中指出CCST和AML关系密切,同属一个家族,这个家族以含有表达HMB45的PEC为特征[3].     

子宫血管周上皮样细胞肿瘤临床病理观察

目的研究子宫血管周上皮样细胞肿瘤的病理学特征、诊断、鉴别诊断和生物学行为。方法对5例子宫血管周上皮样细胞肿瘤进行常规组织学和免疫组织化学（SP法）染色和观察,对患者进行随访,并复习相关文献。结果光镜下5例肿瘤均由透明或嗜酸细胞巢或宽窄不等的细胞索组成,间质有丰富的小血管和程度不等的透明变。免疫组织化学染色示5例瘤细胞均黑色素细胞标记阳性和程度不等的结蛋白和平滑肌肌动蛋白（SMA）阳性,CK和CD10阴性。5例患者现均存活。结论子宫血管周上皮样细胞肿瘤具有较特征性的组织病理及免疫组织化学特点,HMB45阳性对诊断有重要作用。该肿瘤分良性、恶性潜能不能确定和恶性三类,应与透明细胞癌和上皮样平滑肌肿瘤区别。     

Cytoplasmic sequestration phenomena in smooth muscle cells of kidney resistance vessels and epithelioid cells of the juxtaglomerular apparatus

Summary The occurrence of vacuoles in cells of contractile tissues and especially in media cells of resistance vessels has been known for quite some time. Recently, it has been widely accepted that these vacuoles, characteristically lined by a double membrane, result from herniation of one vascular smooth muscle cell into the other as a result of vasoconstriction. In our electronmicroscopic investigations we found double membrane-bounded vacuoles not only in kidney resistance vessels of rats and mice under conditions of vasoconstriction, but also in control animals and animals with maximal renal vasodilation. Part of our observations are compatible with the assumption that such vacuoles arise from a damage of club-shaped, musculo-muscular contacts due to shape changes of media cells during maximal vasoconstriction or vasodilation. However, serial thin sectioning revealed that some of the cytoplasmic vacuoles have no connections with neighbouring cells. This finding and various parallels to the generation of autophagic vacuoles indicate that the so-called herniations may also represent demarcations of large cytoplasmic areas within an individual cell. Irrespective of the origin of these vacuoles, their contents show different stages of deterioration. At later stages, the vacuoles appear to be adjacent, with only one membrane, to the extracellular space, into which they are believed to discharge finally. Cytoplasmic vacuolization has not only been observed in smooth muscle cells, but also in juxtaglomerular epithelioid cells of the afferent arteriole. Here the vacuoles - besides other organelles - also contain secretory granules; it is therefore proposed that autophagic phenomena with final extrusion of cytoplasmic material may be involved in the programmed down-regulation of the granular renin store following inhibition of renin synthesis and secretion.These studies were supported by the German Research Foundation within the Forschergruppe Niere, Heidelberg