乳腺上皮样型管周间质肉瘤病理特征及与叶状肿瘤比较观察

目的 探讨乳腺上皮样型管周间质肉瘤的临床病理特点及与叶状肿瘤的关系。方法 采用HE、特殊染色、免疫组化染色(CK，EMA，S-100蛋白，SMA，Vim，Des，MG，CD34，CD99，CD117，PR，HMB45)对1例乳腺上皮样型管周间质肉瘤与5例叶状肿瘤(良性、交界性各1例，恶性3例)做比较性观察。结果 乳腺管周间质肉瘤(上皮样型)有独特的镜下图像：①显著的多角形(上皮样)细胞绕导管或小管的上皮肌上皮层呈间质性增生，无叶状结构；②组织学模式有袖套状、花冠状、菊形团状、结节状、融合结节状和片状浸润；③瘤细胞形态有：多角形(大、小)、柱状和梭形。多角形细胞呈上皮样形态，异型明显，核分裂象多见(10～30个／10HPF，个别区域达6个／1HPF)，病理性核分裂象易见，在浸润灶内见肿瘤性坏死；④瘤细胞Vim弥漫阳性、EMA灶性阳性、CD99和CD117灶性弱阳性、CD34少数阳性，CK、SMA、S-100蛋白、Des、MG、PR、HMB45均阴性。5例叶状肿瘤均具备叶状结构、间质过度增生、细胞密集(异质性分布)、核分裂象2～10个／10HPF等诊断要素。在3例恶性叶状肿瘤中，2例有极少的上皮样袖套状病灶，2例有梭形细胞袖套状病灶。结论 乳腺上皮样型管周间质肉瘤是一种极罕见的恶性纤维上皮肿瘤亚型，它可能是恶性叶状肿瘤的最早期病变，也可能是一种独特的类型。     

乳腺叶状肿瘤内癌1例临床病理观察

目的探讨乳腺叶状肿瘤内癌的临床病理学特征。方法回顾1例乳腺叶状肿瘤内癌的临床资料、病理学特征及免疫表型,并复习相关文献。结果结合患者30余年前乳腺交界性叶状肿瘤伴导管原位癌病史,对新发的纵隔肿瘤进行免疫组化标记:vimentin弥漫阳性,desmin、p53局灶阳性,Ki-67增殖指数约40%,CK、CK7、CD34、S-100、Myogenin、CK5/6、p63、EMA、SMA、CD117、BCL-2均阴性。结论乳腺叶状肿瘤内癌发病率低,形态特点为叶状肿瘤的背景伴发原位癌或浸润癌,再次复发时可能上皮成分较少或无。诊断时一定要结合原发肿瘤形态,避免误诊。     

子宫Müllerian腺肉瘤伴间质横纹肌样瘤分化1例并文献复习

目的探讨子宫Muellerian腺肉瘤伴间质横纹肌样瘤分化的临床病理特征。方法用光镜、组织化学及免疫组化方法观察其病理组织学表现。结果肿瘤由良性上皮成分和肉瘤性间质成分组成，肉瘤成分过度生长。肿瘤细胞弥漫浸润性分布，细胞大，胞质丰富嗜酸性并可见嗜伊红包涵体。免疫表型：vimentin、CK、NF、CD57、CD99、CgA、Syn阳性，SMA散在阳性，而desmin、EMA、CD10、GFAP、MyoD1、Inhibin—α、HMB45和S-100蛋白阴性。组织化学染色PAS阴性，网状纤维染色显示网状纤维包绕单个或小巢肉瘤性间质细胞。结论子宫腺肉瘤伴间质横纹肌样瘤分化是一种罕见的混合性Muellerian肿瘤，应与子宫内膜间质肉瘤、子宫横纹肌肉瘤和低分化癌等鉴别。     

乳腺交界性叶状肿瘤内小管癌与小叶癌1例并文献复习

目的 探讨乳腺叶状肿瘤内浸润性癌的临床病理学特征、免疫表型、鉴别诊断和预后。方法 收集1例乳腺交界性叶状肿瘤内小管癌与浸润性小叶癌临床资料,采用HE、免疫组化EnVision两步法染色,分析其临床病理学特征并复习相关文献。结果 肿瘤由密集的短梭形间质细胞和单层小管状上皮构成,小管均匀分布于间质,见顶浆分泌,周围肌上皮缺失;间质细胞中度异型,核分裂象多见,另见小叶上皮内瘤变和少量浸润性小叶癌。免疫表型：小管癌ER和PR均强阳性,HER2(0),Ki67增殖指数10%,周围肌上皮标记p63、CK5/6、Calponin均阴性;浸润性小叶癌E-cadherin阴性,p120胞质阳性,ER和PR均强阳性,HER2(0),Ki67增殖指数5%;梭形间质细胞CD34阳性,CD10和SMA均阴性,Ki67增殖指数约30%;无错配修复功能缺陷。结论 交界性叶状肿瘤内小管癌并浸润性小叶癌临床罕见,易漏诊,病理医师需充分取材仔细观察,辅以免疫组化进行鉴别诊断。     

孤立性纤维性肿瘤临床病理学观察

目的探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床、病理组织学、免疫组化特征,提高对该病的认识。方法对7例SFT病例进行大体观察、病理组织学及免疫组化En V ision二步法标记,观察V im、CD34、CD99、bc l-2 Des、SMA、HMBE-1、CK、S-100蛋白在SFT组织中的表达。结果7例SFT分别发生于胸腔、腹腔、纵隔、眼眶及胸壁软组织,临床表现为肿块压迫所致的症状,肿块1.5 cm×1.5 cm×1 cm~20 cm×20 cm×18 cm,光镜下肿瘤细胞由长梭形细胞构成束状、编织状排列,间质内有多少不一的胶原纤维,无特殊的组织构型及形态的多样性为其组织学特点。免疫表型:CD34、V im、CD99均为弥漫强阳性,bc l-2 5例阳性,Des、SMA、HMBE-1、CK、S-100蛋白阴性。结论SFT是一组形态多样、无特殊组织构型及有多少不一胶原纤维的梭形细胞肿瘤,其特殊的免疫表型,有助于对该病的诊断,但SFT的确诊必须结合组织形态和免疫组化共同完成。SFT的生物学行为取决于其组织学形态和肿块的大小及生长方式,完全切除肿块者预后较好。     

乳腺肌上皮癌3例临床病理特征分析

目的探讨乳腺肌上皮癌的临床表现、组织形态学改变和免疫表型特征。方法采用HE及免疫组化Leica BOND-MAX全自动染色仪Bond Polymer Refine Detection法,对3例乳腺肌上皮癌进行染色,并复习相关文献。结果乳腺肌上皮癌的癌组织多由梭形细胞组成,部分病例可见上皮样细胞、浆样细胞和透明细胞,呈实性片状、肺泡样、条索状排列;核分裂象易见;无坏死;可发生腋窝淋巴结转移。免疫表型:3例均表达CK、EMA、CD10、p40、actin、Calponin、p63;2例表达CK5/6、CK14、SMA;1例表达S-100蛋白、D2-40、CAM5.2;CD117、CD34、ER、PR、C-erb B-2/HER-2和desmin均不表达;Ki-67增殖指数为10%~20%。结论肌上皮癌细胞形态多样,结合免疫组化组合标记可做出明确诊断,治疗方式以乳腺癌根治术为主。     

乳腺导管内癌分子分型应用研究

目的 采用免疫组织化学检测方法 对乳腺导管内癌进行分子分型.方法 收集50例乳腺导管内癌存档蜡块,用单克隆抗体CK5/6、CK8、CK18、34βE12、p63、S-100、SMA、CD10、CD117、EGFR、ER、PR和HER2进行免疫组织化学EnVision法染色,按照免疫表型分为5种类型:腺腔A型(ER+/PR+/HER2-)、腺腔B型(ER+/PR+/HER2+)、正常乳腺样型(ER-/PR-/HER2-且不表达基底/肌上皮标记及EGFR)、HER2过表达型(ER-/PR-/HER2+)和基底细胞样型(ER-/PR-/HER2-,且至少表达一种基底型角蛋白和(或)肌上皮标记物或EGFR).结果 腺腔A型16例(32%),腺腔B型19例(38%),HER2过表达型13例(26%),基底细胞样型2例(4%),无正常乳腺型.2例基底细胞样型,均表达CK5/6、CD117,例1同时表达SMA,例2表达CK8、CK18、34βE12、S-100,均为高级别导管内癌.结论 乳腺导管内癌可按免疫表型进行分子分型,部分导管内癌具有与基底细胞样癌相同的免疫表型,可能是基底细胞样癌的前驱病变,其诊断依赖于免疫组化检测.     

卵巢黏液性囊腺瘤合并性索-间质肿瘤的临床病理观察

目的 探讨卵巢黏液性囊腺瘤合并性索-间质肿瘤(MCSC)的临床病理特点、组织起源及其诊断和鉴别诊断.方法 对1例MCSC进行组织形态学、免疫组化观察,并复习相关文献.结果 患者发现盆腔肿物1年,无明显不适及内分泌症状.大体上肿瘤为多房囊性,囊内壁可见散在的实性结节,镜检肿瘤由黏液性囊腺瘤和性索间质肿瘤两种成分组成,前者囊壁衬覆良性肠型黏液柱状上皮,性索间质成分瘤细胞呈小管状、条索状、梁状排列,局部有黄素化,两种成分混合存在,未见移行.免疫组化:性索成分瘤细胞呈Melan A弥漫强阳性;αInhibin和CD56局灶阳性;并见CKpan、vimentin、ER及PR的阳性表达;而EMA、CEA、CD99、S-100、CgA、Syn、nestin及SMA均阴性;Ki-67指数小于5%.结论 MCSC是罕见的卵巢肿瘤,性索间质成分可能是黏液性囊腺瘤囊壁内间质的一种反应性增生.诊断时需与类癌、伴异源成分的支持-间质细胞瘤、畸胎瘤等鉴别.     

原发性肾脏滑膜肉瘤临床病理及分子遗传学分析

目的探讨原发性肾脏滑膜肉瘤的临床病理特征、诊断及鉴别诊断。方法对1例罕见的原发性肾脏滑膜肉瘤进行光镜观察和免疫组化染色,并用RT-PCR方法在石蜡包埋组织中检测SYT-SSX融合mRNA表达,结合文献分析讨论。结果肿瘤由核分裂活跃的单形的梭形细胞组成,呈交织的束状或实性排列,部分区域呈血管外皮瘤样图像,未见上皮分化成分;囊性区囊内衬核分裂不活跃的多边形的鞋钉样上皮。免疫表型瘤细胞vimentin、CD99、bcl-2、S-100均阳性,Ki-67强阳性;CK(AE1/AE3)、EMA、SMA、CD117、CD34、p53、ER、PR均阴性。RT-PCR方法检测到肿瘤组织中有SYT-SSXmRNA表达,对照组SYT-SSX无表达。患者术后5月内死亡。结论原发性肾脏滑膜肉瘤罕见,需要与肾脏其他一些梭形细胞肿瘤相鉴别,分子遗传学检测SYT-SSX融合mRNA有助于其诊断和鉴别诊断。     

肝脏钙化性巢状间质-上皮肿瘤1例报道及文献复习

目的探讨肝脏钙化性巢状间质-上皮肿瘤的临床病理学特征、免疫表型、诊断和鉴别诊断。方法对1例肝脏的钙化性巢状间质-上皮肿瘤进行大体、组织形态学和免疫组化检查,结合文献分析其临床生物学行为。结果组织学上,肿瘤主要由梭形到上皮样细胞呈巢状排列伴周围明显的促纤维间质,梭形细胞胞质淡染或嗜酸性,核杆状;胞界不清,上皮样细胞胞质透亮,细胞界限清楚,核圆形位于中央,核分裂象1个/10HPF;可见骨化和微囊形成。免疫组化染色显示肿瘤细胞表达CKpan、vimentin和WT1胞质阳性,S-100蛋白仅在骨化区域表达,肿瘤间质细胞表达SMA,而AFP、HepPar1、CK19、CD56、CD34、CD117、desmin、myogenin、ER、PR、CgA、Syn均表达阴性,Ki-67增值指数约5%。结论肝脏钙化性巢状间质-上皮肿瘤是一种罕见的肝脏原发性低度恶性肿瘤,应与肝脏其他侵袭性恶性肿瘤鉴别。     

钙化性纤维性肿瘤4例临床病理学分析

目的 探讨钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的病理形态学特点、免疫组化表达及鉴别诊断.方法 对4例CFT进行免疫组化检测并文献复习.结果 本组患者年龄19～33岁,发病部位分别为胃、纵膈、大网膜及肠系膜根部.镜下可见在大量胶原化的纤维组织内伴有钙化或砂砾体形成,淋巴细胞、浆细胞散在浸润.免疫组化标记瘤细胞表达vimentin,而CD117、CD34、CD99、desmin、S-100蛋白、SMA、actin、NFP、ALK1、ER、PR、CK、BCL-2及Ki-67均阴性.结论 CFT是一种极少见的良性纤维性肿瘤,其诊断需要结合年龄、组织学形态及免疫组化等特点,还需与钙化性腱膜纤维瘤、钙化性肉芽肿、纤维瘤病、胃肠道间质瘤、炎性肌纤维母细胞瘤、结节性筋膜炎和淀粉样瘤等相鉴别.     

CD34, CD117, and Ki-67 expression in phyllodes tumor of the breast: an immunohistochemical study of 33 cases

Phyllodes tumors (PTs) of the breast are biphasic lesions, comprising an epithelial component set within a neoplastic spindle-celled stroma. These tumors have been classified as benign, borderline, and malignant based on a combination of histological criteria, including stromal cellularity, nuclear pleomorphism, mitotic rate, stromal overgrowth, and margin appearance. The aim of this study was to evaluate the expression of CD34, CD117 (c-kit), and Ki-67 in PT of the breast and attempt to correlate the staining pattern with tumor grade by morphology. Immunohistochemical expression of CD117, CD34, and Ki-67 was studied on formalin-fixed, paraffin-embedded archival tissue material from 33 cases of PT. Histologically, there were 21 benign, 6 borderline, and 6 malignant (high-grade) tumors. All 6 histologically malignant PTs were positive for CD117 (100%), but only 1 marked with CD34 (16.7%). Borderline PTs frequently coexpressed CD34 and CD117 (66.7%). The benign PTs, on the other hand, most commonly (52.4%) showed a CD34(+)/CD117(-) immunoprofile with 33.3% cases coexpressing the markers: that is, CD34(+)/CD117(+). Although most benign PTs (80.6%) showed a Ki-67 of <2%, a few cases showed slightly higher proliferation indices. This study indicates that CD34 and CD117 are differentially expressed in benign and malignant PTs. These markers, therefore, in combination, may be used as an adjunct to morphology in the subclassification of PTs.     

An immunohistochemical study of metaplastic spindle cell carcinoma, phyllodes tumor and fibromatosis of the breast

The diagnosis of metaplastic (sarcomatoid) carcinoma (MSC) of breast often requires immunohistochemistry with a cytokeratin (CK) panel to distinguish them from phyllodes tumors (PT), primary sarcomas, and fibromatoses. CK staining may be heterogeneous in metaplastic carcinomas. The aim of the study was to investigate the theory that MSCs show evidence of myoepithelial differentiation and to evaluate immunohistochemical markers that may be helpful in distinguishing MSCs from PT and fibromatosis. We reviewed histology and performed immunohistochemistry for AE1/AE3, 34betaE12, CK5 and CK14, Cam5.2, CK7 and CK19, epithelial membrane antigen (EMA) (B55), smooth muscle actin (SMA), S100, desmin, vimentin, CD31, CD34, and bcl-2 on paraffin-embedded tissue from 18 MSCs, 26 PTs, and 8 fibromatoses. We assessed staining by using a semiquantitative method. Sarcomatous areas in MSCs were positive for 34betaE12 in 11 cases; for SMA in 10; for CK5 in 7; for CK14 in 6; for Cam5.2, AE1/AE3, and S100 in 5; and for CK7 and CK19 in 3. No CK expression was seen in stromal areas in PT or in fibromatoses. CD34 and bcl-2 were more frequently expressed in spindle cell areas in PTs (18 and 12 of 26, respectively) than in MSCs (0 and 2 of 18, respectively). MSCs show strong evidence of myoepithelial differentiation. CD34 and, to a lesser extent, bcl-2 positivity in PTs may be helpful in differentiating these two lesions from MSCs, particularly in small biopsies, because CK staining in MSCs may be heterogeneous. In our hands, 34betaE12 was the CK most frequently expressed in sarcomatoid areas in MSCs.     

小肠间质瘤的免疫组织化学特征其意义

目的探讨小肠间质瘤的临床病理诊断及免疫组织化学特征。方法收集我校附属医院临床病理科6年来对外科手术切除的小肠肿瘤标本,光镜观察、免疫组织化学方法检测Vimentin、desmin、S-100、actin、CD117、CD34和SMA的表达。结果13例肿瘤中良性3例,潜在恶性4例,恶性6例;其中黏膜下生长3例,浆膜下生长5例,肠系膜处生长2例,肌壁内生长3例,常见症状是上腹部肿块和上消化道出血,免疫表形特征为:CD11713例(100%)胞质阳性表达;CD3410例阳性(76.9%)表达,S-100蛋白2例(15.4%)呈局灶表达,10例SMA阴性表达。结论小肠间质瘤发生率较高,多为恶性,有必要和肠的雪旺细胞瘤、平滑肌瘤相区别,CD117、CD34可作为诊断小肠间质瘤免疫标记物,而肿瘤大小、有无出血和坏死、核分裂像等均可作为良恶之判断的参照指标。     

Malignant myoepithelioma of the breast: a case report and review of literature

In this article, we described a malignant myoepithelioma of the breast (MMB) in a 69-year-old woman. Breast cancer derived from myoepithelial cells is very rare, usually benign. The diagnosis of MMB based on histological and immunohistochemical finding. In this case, the author diagnosed the tumor as MMB, because tumor tissues were immunopositive for 34βE12, P63, SMA, S-100, CD10, E-Cad and Ki-67, and immunnegative for CK5/6, desmin, ER, PR and C-erbB-2, because tumor tissue showed invasive growth and local hemorrhage or necrosis, suggesting malignant, and also because there was a transition between the tumor cells and hyperplastic myoepithelium of non-tumorous ducts. The patient’s postoperative recovery is smooth and regular following of patient is essential.     

Differential diagnosis of gastrointestinal stromal tumor and other spindle cell tumors in the gastrointestinal tract based on immunohistochemical analysis

To confirm the usefulness of an immunohistochemical panel of antibodies for KIT (c-kit/CD117), CD34, desmin, smooth-muscle actin (SMA), h-caldesmon (HCD), S-100 protein, neuron-specific enolase (NSE), and beta-catenin, 297 mesenchymal and peripheral nerve-sheath tumors of the gastrointestinal tract and intra-abdominal locations including 211 gastrointestinal stromal tumors (GISTs), 12 leiomyomas, 18 leiomyosarcomas, 17 solitary fibrous tumors (SFTs), 14 schwannomas, and 25 desmoid-type fibromatoses (DTFs) were analyzed immunohistochemically. Consistent (100%) immunoreactivity for KIT, CD34, desmin and S-100, and nuclear accumulation of beta-catenin were detected in GISTs, SFTs, smooth-muscle tumors, schwannomas, and DTFs, respectively. Immunoreactivity for SMA, HCD, and NSE was observed in a wide range of these tumors. In addition, 418 bone and soft tissue tumors were enrolled in this study for KIT immunostaining. As a result, a limited number of these tumors were KIT positive, including synovial sarcoma that showed morphological similarity to GISTs. These findings suggest that KIT, CD34, desmin, S-100, and beta-catenin are key markers for clinical diagnosis of GISTs and other spindle cell tumors that may involve the gastrointestinal tract, whereas SMA, HCD, and NSE have only limited value.     

细胞周期蛋白D1、Ki-67和bcl-2的表达与CD117阳性的胃肠道间质瘤生物学行为的关系

目的 检测细胞周期蛋白D1（cyclin D1）、Ki-67和bcl-2在胃肠道间质瘤（GIST）中的表达,探讨它们在GIST发生、发展中的作用及临床病理意义。方法 59例手术切除GIST标本进行CD117、CD34、平滑肌肌动蛋白（SMA）、结蛋白、S-100、cyclin D1、bcl-2和Ki-67免疫组织化学染色,同时进行病理形态学观察包括形态学类型、肿瘤大小、坏死和核分裂象。所有病例随访2～9年。所有数据进行单因素、多因素和相关分析。结果 随访40例患者一直健在,15例患者死于GIST,4例患者死于其他原因。统计学分析显示肿瘤直径〉5cm、有坏死、核分裂象在每50个高倍视野〉5个、Ki-67增殖指数（LI）〉5％、cyclin D1和bcl-2免疫组织化学染色强阳性都可以作GIST患者手术后的预测指标,且具有统计学意义;核分裂象和Ki-67增殖指数是独立的预测指标;Ki-67 LI≥5％和核分裂象≥5／50 HPF呈正相关（r=0．532,P〈0．01）;cyclin D1与bcl-2强阳性表达呈正相关（r=0.273,P〈0．05）。结论 肿瘤大小、坏死、核分裂象、cyclin D1、Ki-67增殖指数和bcl-2可作为GIST患者临床预测指标;核分裂象和Ki-67增殖指数可作为独立的预测指标;cyclinD1与bcl-2呈明显相关性,Ki-67免疫组织化学染色可以代替核分裂象作为一项很有用的预测指标。     

Steroid hormone receptor in pleural solitary fibrous tumours and CD34+ progenitor stromal cells

Solitary fibrous tumours (SFT), originally described in the pleura, were subsequently recognized in numerous extrapleural sites. This suggests that a common stem cell, present in various organs and tissues, may be at the origin of SFT and that specific factors may be involved in the proliferation of such cells. Recently it has been described that steroid hormone receptors, progesterone receptors in particular, are expressed by extrapleural SFT. In addition, progesterone may participate as growth factor in many CD34(+) stromal neoplasms, which express low levels of the hormone receptors. The present study analysed the expression of androgen (AR), oestrogen (ER) and progesterone (PR) receptors in a series of 32 pleural SFT, 10 mesotheliomas and in reactive tissue of chronic pleuritis. ER and AR were never expressed by SFT or by chronic pleuritis, whereas PR were demonstrated in 2/16 "large" (>8 cm) and in 6/16 "small" (< or =8 cm) pleural SFT (all expressing CD34, bcl-2 and CD99). PR(+) SFT had a significantly higher proliferative activity (p = 0.04) (Ki-67 mean value 6.5%) and lower p27(kip1) (mean value 51.5%) expression than the PR(-) cases (Ki-67 mean value 3.81% and p27(kip1) mean value 57.86%). One of the cases expressing a high level of PR (80%) recurred 1 year after first surgery and the recurrence was PR(+) as well, but with a lower percentage of nuclear receptor expression (12%). In addition, in chronically inflamed subserosal tissue, a subpopulation of CD34(+) endothelial and interstitial dendritic cells was identified, which also expressed PR. These findings suggest that the CD34(+) submesothelial interstitial dendritic cells, activated during reactive processes, may be the stem cells that give rise to SFT, and that progesterone might participate in the growth of SFT through modulation of its specific receptors.