乳腺腺肌上皮瘤临床病理观察及文献复习

目的探讨乳腺腺肌上皮瘤（adenomyoepithelioma,AME）的形态学及免疫组化表型特征。方法对2例乳腺AME进行临床病理学分析和免疫组织化学研究。结果肿瘤由增生的腺上皮和肌上皮组成。腺上皮CK、EMA、ER、PR（＋）;肌上皮CK（-）,S-100蛋白、SMA、actin（＋）、EMA局部弱（＋）,GFAP、desmin（-）。结论乳腺AME是一种具有独特病理学特征的良性肿瘤,由腺上皮和肌上皮共同增生而成。熟悉该肿瘤的组织学和免疫表型特点对鉴别诊断有重要意义。     

15例涎腺肌上皮瘤临床病理学分析

目的探讨涎腺肌上皮瘤的临床病理学特点、诊断及鉴别诊断。方法对15例涎腺肌上皮瘤的临床特点、组织学形态、免疫表型进行观察,并结合文献复习。结果涎腺肌上皮瘤主要发生在腮腺、上腭处,瘤细胞呈梭形、浆细胞样、上皮样、透明细胞样,细胞无明显异型,呈岛、片、网状结构分布,瘤细胞间可见均质红染的玻璃样物或大量黏液成分,免疫表型:CK5/6、S-100、p63、SMA、calponin。结论涎腺肌上皮瘤是少见肿瘤,细胞类型多,排列方式以片状为主,很少见到管腔样结构,确诊主要依靠其细胞形态以及排列方式,需与肌上皮癌、多形性腺瘤等鉴别。     

涎腺肿瘤的病理诊断

1 涎腺肿瘤概述 正常涎腺导管上皮的cytokeratin、CEA（多克隆）、EMA免疫组化染色为阳性表达,S-100蛋白阴性表达,肌上皮和基底细胞为cytokeratin、p63阳性表达,S-100蛋白和GFAP表达常不确定,calponin（图1）和actin只表达于肌上皮.但是在涎腺肿瘤中,腺上皮和非腺上皮可出现异常的免疫表型,如actin在肿瘤性肌上皮可呈阴性表达,以及在正常涎腺阴性表达的CD117在肿瘤性腺上皮常阳性表达（图2）.此外,正常的涎腺导管细胞常分泌中性黏液（PASD阳性）、肌上皮常分泌酸性间质型蓝色黏液（alcin阳性）和基膜样物质（PASD阳性）.[第一段]     

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。     

近端型上皮样肉瘤临床病理研究

目的 探讨近端型上皮样肉瘤(PES)的临床病理特点、诊断及鉴别诊断依据.方法 收集5例PES患者资料,免疫组织化学EnVision法染色.第一抗体选用细胞角蛋白(CK)、波形蛋白、上皮细胞膜抗原(EMA)、CD34、β-catenin、S-100蛋白、平滑肌肌动蛋白(SMA)、肌调节蛋白(MyoD1)、结蛋白、HMB45、CK7及CK20,观察和分析其临床病理学形态及免疫表型特征.结果 5例PES中女1例,男4例;发病年龄19-46岁,发生部位分别为:会阴部2例,下腹部、髂前上棘和臀部各1例,均表现为进行性增大的无痛性单发肿块.光镜下肿瘤细胞呈结节状排列,浸润性生长,瘤细胞大部分由相对独特的上皮样细胞组成,胞质丰富,嗜酸性;核卵圆形,肿瘤中心常见坏死.免疫组织化学染色示5例瘤细胞均表达波形蛋白,4例表达CK、EMA,3例表达β-catenin、CD34,1例表达S-100蛋白;而SMA、MyoD1、结蛋白、HMB-45、CK7及CK20均阴性.结论 根据PES组织学和病理学特征,结合免疫组织化学染色结果可以做出明确诊断.     

乳腺腺肌上皮瘤5例并临床病理分析

目的探讨乳腺腺肌上皮瘤(adenomyoepithelioma,AME)的临床病理学和免疫表型特征。方法采用HE及免疫组化EnVision两步法对5例AME进行染色,并复习相关文献。结果 5例患者均为女性,年龄45~63岁(平均54岁)。镜下见肿瘤由增生的腺上皮和肌上皮组成,以肌上皮增生为主,多为梭形或透明肌上皮细胞。伴癌的AME中可见肿瘤呈浸润性生长,细胞丰富,异型性明显,核分裂象增多,肿瘤内有坏死。免疫表型:5例肿瘤腺上皮CK强阳性,EMA、ER、PR、SMA、p63、CD10、Calponin、CK5/6均阳性,肌上皮CK弱阳性,ER和PR均阴性。结论 AME为乳腺少见的良性肿瘤,由腺上皮和肌上皮共同增生形成。伴癌的AME更罕见,其恶性成分多为肌上皮,肿瘤转移少,转移者预后差。     

乳腺非典型腺肌上皮瘤1例

本文报道1例42岁女性乳腺腺肌上皮瘤。患者查体右乳见一明显肿块, 质稍硬, 无触压痛, 皮肤无红肿、破溃, 双侧乳头无凹陷, 无溢血, 可见少量溢液。镜下见肿瘤呈多结节性生长, 导管上皮细胞和肌上皮细胞增生, 并且以肌上皮细胞增生更明显, 局灶有浸润性生长模式, 肿瘤细胞胞质丰富、透明, 细胞轻至中度异型, 细胞核大小不等, 可见少量核分裂象(<3个/10 HPF)。免疫组织化学染色:腺上皮细胞表达细胞角蛋白(CK)7;肌上皮细胞表达CK5/6、S-100蛋白、p63、雌激素受体;孕激素受体、突触素、Calponin、肌特异性肌动蛋白、CK10/13、IDH2阴性, Ki-67阳性指数约5%。本例诊断主要结合病理形态学特点及免疫组织化学染色。     

涎腺嗜酸细胞性脂肪腺瘤二例

报道2例罕见的发生于腮腺的涎腺嗜酸细胞性脂肪腺瘤。大体观察:2例均包膜完整,1例呈多结节状,1例呈哑铃状。镜下观察:2例肿瘤均被覆薄的纤维性包膜,由不同比例的脂肪组织和大小不等的嗜酸性细胞巢混合构成。免疫组织化学染色:2例嗜酸性细胞均表达人低分子量细胞角蛋白,部分表达细胞角蛋白(CK)7、CK5/6、CK19、p63、上皮细胞膜抗原(EMA),不表达S-100蛋白、Calponin、DOG1,Ki-67阳性指数约1%~2%。2例分别随访4和5个月,均未见复发。     

肾脏上皮样血管平滑肌脂肪瘤的病理观察

目的对肾脏上皮样血管平滑肌脂肪瘤（epithelioid agiomyolipoma,EAML）的病理诊断、鉴别诊断和预后进行分析。方法2例肾脏EAML（其中1例为复发病例）,复习其临床资料,病理学检查包括常规病理学、免疫组织化学和超微结构,并进行随访。结果光镜下肿瘤均主要由具有多形性和不典型性的上皮样细胞组成,部分区域有明显的血管周上皮样排列;可见出血和坏死;并可见静脉内瘤栓;淋巴结内可见上皮样肿瘤细胞累及。免疫组织化学肿瘤细胞（包括淋巴结内肿瘤）HMB45、平滑肌肌动蛋白（SMA）、神经元特异性烯醇化酶（NSE）和波形蛋白弥漫阳性;S-100、melanpan和CD68散在阳性;而上皮细胞膜抗原（EMA）、AE1／AE3、CK7、CD117、肌肉特异性肌动蛋白（MSA）、结蛋白、白细胞共同抗原（LCA）、CD20、CIM5RO、CD30、CD15、嗜铬素（CgA）、突触素（Syn）、bcl-2、雌孕激素受体（ER、PR）和p53均为阴性。电镜检查可见一些肿瘤细胞内有黑色素小体样的致密颗粒、肌丝、密体,肿瘤细胞外可见不连续的基膜。2例患者手术后10个月状态良好,无肿瘤局部复发和转移征象。结论血管周上皮样排列、寻找经典血管平滑肌脂肪瘤的结构和肿瘤细胞表达HMIM5和SMA对于诊断和鉴别诊断至关重要。而细胞的不典型性、出血坏死和核分裂象可能只表明肿瘤的恶性潜能：淋巴结受累、肾静脉瘤栓不是恶性的诊断依据：只有远处转移才是恶性的证据。     

乳腺上皮-肌上皮性肿瘤4例临床病理学分析

目的：探讨乳腺上皮-肌上皮性肿瘤(epithelial-myoepithelial tumor of breast)的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对4例乳腺上皮-肌上皮性肿瘤的临床特点、组织形态学及免疫组织化学结果进行分析,并复习相关文献。结果：患者：男性1例,女性3例,平均年龄51岁(27~63岁)。4例肿瘤直径1.5~3.0 cm(平均2.0 cm),无包膜,切面灰白色。显微镜下可见肿瘤由双相增生的肌上皮细胞和腺上皮细胞构成,肌上皮细胞环绕腺上皮细胞构成特征的套管结构。免疫组织化学染色,腺上皮细胞表达CK8/18、CK7,肌上皮细胞表达p63、Calponin、CK5/6。1例诊断为腺肌上皮瘤(adenomyoepithelioma,AME),3例诊断为伴有癌的腺肌上皮瘤(恶性腺肌上皮瘤, malignant adenomyoepithelioma,MAME)。结论：乳腺上皮–肌上皮性肿瘤是少见的肿瘤类型,需与导管内乳头状瘤、化生性癌等鉴别。     

Carcinosarcoma of the urinary bladder: Expression of epithelial markers and different expression of heat shock proteins between epithelial and sarcomatous elements

A case of carcinosarcoma composed of both adenocarcinoma and saarcomataus elements in the non-trigone region of the urinary bladder is presented. The epithelial element was a well to pooriy differentiated adenocarcinome with focal squamous metaplasia. The sarcomatous elements disclosed spindle cell sarcoma with focal epltheliold pattern and myxold change In the stroma, together with chondrosarcomatous and rhabdomyosarcomatous elements. By Immunohistochemical examination, not onty the carcinoma element but also the sarcomatous elements showed a positive lmmunoreaction for cytokeratin (CK), epithelial membrane antigen (EMA) and carcinoembryonic antigen. Some population of sarcomatous elements expressed smooth muscle actin and muscle specific actin (MSA) and a limited portion of epitheliold area showed a positive Immunoreaction for desmin, MSA and myoglobin, Indicating leiomyosarcomatous and rhabdomyosarcomatous differentiation, respectively. Unexpectedly, tumor cells In the chondrosarcomatws element revealed a simultaneous positvity of CK and EMA as well as S-100 protein. Both epithelial and sarcomatous elements showed an Intensive positive Immunoreaction for p53 and heat shock protein (HSP) 70. However, HSP27 and HSPGO were detected in most epitheilal elements and only in a small number of tumor cells in the sarcomatous area. These findings indicate that sarcomatous elements, Including heterologous elements, may derive from epithelial elements with partial or complete loss of epithelial features, and different factors other than p53 and HSP7O may associate wtth the morphological alteration of carcinoma.     

鼻腔鼻窦炎性肌纤维母细胞瘤的临床病理学分析

目的 探讨鼻腔鼻窦炎性肌纤维母细胞瘤(IMT)的临床病理学特征、免疫表型及超微结构特点.方法 分析5例鼻腔鼻窦IM'I'的临床和病理形态学特点,用波形蛋白、肌特异性肌动蛋白(MSA)、平滑肌肌动蛋白(SMA)、钙调节蛋白(calponin)、h-钙结合蛋白(h-caldesmon)、结蛋白、间变性淋巴瘤激酶(ALK)、纤连蛋白(FN)、CK、S-100蛋白、Ki-67等抗体进行免疫组织化学(SP法)标记,取2例做透射电镜观察.结果 患者发病年龄28～62岁(平均年龄43岁),男性2例,女性3例,临床表现有鼻堵、流涕,面部疼痛、肿胀,牙疼、溢泪等,1例表现为鼻出血.5例均有复发,4例复发5次以上,1例淋巴结转移,3例死亡.组织学改变:肿瘤细胞排列呈编织状、束状、部分杂乱无序,细胞呈长梭形,有嗜酸性胞质,异型性不明显,核分裂象较少,间质内有较多淋巴细胞、浆细胞浸润,部分间质黏液变性,富于血管,部分区可见透明样变的胶原束.3例多次复发后肿瘤细胞异型性明显,核分裂象增多(平均5～6个/10 HPF),可见小灶状坏死,问质内炎性细胞减少,呈肉瘤改变.免疫组织化学标记显示:5例瘤细胞均弥漫表达波形蛋白,不同程度表达SMA、MSA、calponin和FN,结蛋白仅1例弱阳性,h-caldesmon、ALK、S-100蛋白、CK均为阴性,反复复发后Ki-67示瘤细胞增殖指数增高.电镜显示:瘤细胞胞质内可见较多的粗面内质网,部分瘤细胞的胞质内可见密体和密斑的肌丝束,细胞外基质中见多量的胶原纤维.结论 鼻腔鼻窦IMT易复发,有局部侵袭性,反复复发后可恶变及转移,病死率高,预后差.完整切除肿瘤是治疗的关键.     

Nasal myoepithelioma removed through endonasal endoscopic surgery: a case report

Myoepithelioma is a rare neoplasm that can occur in either the major or minor salivary gland and accounts for less than 1% of salivary gland neoplasms. We report a rare case of a nasal myoepithelioma that originated from the nasal inferior turbinate. The tumor, measuring 50 × 30 × 20 mm, was in the right nasal cavity and had a necrotic surface. We removed the tumor through endonasal endoscopic surgery. The tumor had spindle-shaped cells and was positive for cytokeratin, (AE1/AE3), vimentin, S-100β, and MIB-1 but was negative for CD34, desmin, neuron-specific enolase, and synaptophysin. Slight immunoreactivity for smooth muscle actin was noted in some tumor cells. There has been no evidence of tumor recurrence in the 18 months following surgery.     

Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: an immunohistochemical comparison of 34 cases.

Endometrial stromal sarcoma (ESS), uterine cellular leiomyoma (UCL), and uterine leiomyosarcoma (ULS) are composed mainly of spindle cells that express similar antigens such as desmin, smooth muscle actin (SMA), and muscle-specific actin (MSA). The differential diagnosis of an ESS versus a uterine smooth muscle tumor or an extrauterine spindle cell sarcoma can be problematic based solely on clinical presentation, histologic assessment, or routine immunohistochemistry. Recently, we reported that normal endometrium, but not myometrium, as well as five cases of ESS, were positive for CD10. We now report the results of CD10 immunohistochemistry in an additional 11 cases of ESS (total 16 cases), 10 cases of UCL, and nine cases of ULS. CD10 immunoreactivity was detected in 16 of 16 cases of ESS (100%) as compared to only 2 of 10 cases of UCL (20%) and none of nine cases of ULS (0%). We compared the utility of CD10 immunoreactivity with that of desmin, SMA, MSA, estrogen receptor (ER), and inhibin in these tumors. Although the majority of cases of UCL and ULS were positive for SMA, MSA, and desmin, a substantial portion of cases of ESS were also positive for SMA, MSA, and desmin. We conclude that in combination with SMA, MSA, and desmin, CD10 is a useful immunohistochemical marker in the differential diagnosis of ESS versus UCL or ULS.     

Carcinoma ex pleomorphic adenoma: pathologic analysis of 73 cases.

Pathologic factors of predictive value for carcinoma ex pleomorphic adenoma (CXPA), an aggressive salivary gland malignancy, are poorly defined. Because residual mixed tumor may be relatively inconspicuous and various carcinoma subtypes are encountered, misdiagnosis is common. To describe the pathologic features and identify potential prognostic factors, we retrospectively examined 73 cases of CXPA of the major salivary glands treated at Mayo Clinic. Paraffin section immunostaining for keratins (AE1/AE3, CK7, CK20), epithelial membrane antigen, carcinoembryonic antigen, vimentin, actin, S-100 protein, glial fibrillary acidic protein, and p53 and c-erbB-2 oncoproteins was performed in 69 cases. DNA content and proliferation indices were determined by digital image analysis of Feulgen- and MIB-I-stained sections, retrospectively. Survival was calculated by the Kaplan-Meier method, and prognostic variables were analyzed with the log-rank test. The carcinoma component was predominant in 82% of tumors. Adenocarcinoma not otherwise specified (31 cases) and salivary duct carcinoma (24 cases) were the most frequent histologic subtypes. Sixty-two tumors were high grade (Broders 3 or 4). Residual mixed tumor was extensively hyalinized in 54 cases. Pathologic features significantly associated with overall survival included pathologic stage (P =.009), tumor size (P =.012), grade (P =.005), proportion of carcinoma (P =.004), extent of invasion (P =.002), and proliferation index of carcinoma (P =.03). Of 4 patients with intracapsular (noninvasive) carcinoma, none had an adverse outcome. The immunohistochemical profile of CXPA included positive staining reactions in the malignant component for AE1/AE3 in 97% of cases, CK7 in 94%, epithelial membrane antigen in 86%, carcinoembryonic antigen in 75%, vimentin in 52%, and S-100 protein in 29%. Expression of p53 and c-erbB-2 oncoproteins was detected in 41% and 30% of the carcinomas, respectively, but neither was associated with decreased survival. High-grade salivary adenocarcinoma that is difficult to classify should raise the suspicion of possible CXPA. Intracapsular carcinoma has a benign clinical course. Significant prognostic factors in CXPA include tumor stage, grade, proportion of carcinoma, extent of invasion, and proliferation index.     

Malignant mixed müllerian tumor of the ovary. Report of a case studied by immunohistochemistry.

A case of malignant mixed müllerian tumor of the ovary in a 57-year-old woman is reported along with the results of an immunohistochemical study. The tumor, measuring 16 x 10 x 9 cm, was composed predominantly of adenocarcinoma with a smaller amount of anaplastic carcinoma as an epithelial component and chondrosarcoma, liposarcoma, fibrosarcoma and rhabdomyoblasts as mesenchymal elements. Immunohistochemistry using paraffin sections demonstrated cytokeratin (CK) and epithelial membrane antigen (EMA), generally regarded as epithelial markers, not only in the epithelial component but also in chondrosarcoma cells. Vimentin and desmin, generally regarded as mesenchymal markers, were exhibited partly in carcinoma cells as well as in mesenchymal elements. Positive staining for S-100 protein was obtained not only in chondrosarcoma and liposarcoma cells, but also partly in adenocarcinoma cells. This intricate immunohistochemical picture reflected the histologic findings. It is noteworthy that both carcinoma cells and chondrosarcoma cells demonstrated simultaneous expression of CK, EMA, vimentin, desmin and S-100 protein. This somewhat unusual antigen expression by tumor cells may indicate a change in the nature of tumor cells due to microenvironmental factors.     

Superficial malignant peripheral nerve sheath tumor: a rare and challenging diagnosis

We reviewed the clinicopathologic features of 5 cases of malignant peripheral nerve sheath tumor (MPNST) manifesting in superficial locations associated with cutaneous neurofibromas (4 cases) or superficial peripheral nerve (1 case). Four cases had spindle cell morphologic features and were at least focally positive for S-100 protein, whereas the associated benign neural elements had more extensive S-100 immunoreactivity. The single epithelioid case was diffusely and strongly positive for S-100 protein. Melanoma markers, epithelial membrane antigen, glial fibrillary acidic protein, neurofilament, pancytokeratin (AE1/AE3), CD34, smooth muscle actin, and desmin were negative in all cases. There were no local recurrences, but 3 patients died of metastatic disease within 2 to 30 months (median, 21 months). MPNSTs can occur in a superficial location and may have an aggressive clinical course. Immunohistochemical markers are helpful in excluding other lesions in the differential diagnosis. However, identification of a benign precursor or origin from a nerve may be the most definitive way to properly classify these rare lesions.     

Malignant myoepithelioma with a squamous epithelial component in the mammary gland of a cynomolgus monkey

A solid mass arising from the mammary gland was found in a 7-year-old female cynomolgus monkey. Histologically, the mass consisted of 2 components: spindle-shaped or ovoid sarcomatous cells and squamous epithelial cells. Metastatic nodules noted in the lung, liver and the gallbladder had the same histological features as the mammary mass. Immunohistochemistry revealed that the sarcomatous cells were positive for alpha-smooth muscle actin (alpha-SMA), vimentin, calponin, S-100 protein, epithelial membranous antigen (EMA), cytokeratin (large spectrum) and cytokeratin 14 (CK 14) in the cytoplasm, and p53, erbB-2 and progesterone receptor in the nuclei, but negative for desmin and estrogen receptor. The squamous epithelial cells were positive for EMA, cytokeratin (large spectrum) and CK 14, but negative for the rest. Both sarcomatous and squamous epithelial components were negative for glial fibrillary acidic protein (GFAP). Based on histological and immunohistochemical features, the present case was diagnosed as a malignant myoepithelioma with a squamous epithelial component in the mammary gland with distant metastases.     

Myoepithelial carcinoma in pleomorphic adenoma of salivary gland type, occurring in the mandible of an infant

A rapidly growing mandibular tumor occurred in a 17 month old female infant. Tumor outgrowth showing a periosteal reaction was radiographically seen on the lower surface (base) of the mandible. Under the biopsy diagnosis of osteosarcoma, high-dose chemotherapy with methotrexate was performed, resulting in little effect. The right hemiman-dibulectomy specimen disclosed intraosseous infiltrative growth of pleomorphic adenoma of salivary gland type, associated with chondroid stroma and reactive bone formation. The highly proliferative small-sized cells retained immunohistochemical features of myoepithelial cells, with positive reactivity of cytokeratin, vimentin, S-100 protein, a-smooth muscle actin, epithelial membrane antigen, CA15–3, type IV collagen, laminin and p53 protein. No heterotopia of the salivary gland was identified within the bone tissue. The tumor recurred 2 months later. Due to uncontrollable local growth, the patient died 8 months after operation. At autopsy, reactive ossification was closely associated with malignant myoepithelial proliferation. No distant metastasis was noted. This osteosarcoma-like tumor can be regarded as myoepithelial carcinoma in pleomorphic adenoma, originating from intramandibular heterotopic salivary gland tissue.     

Malignant myoepithelioma of the parotid gland: cytologic and histologic features

A case of malignant myoepithelioma of the parotid gland in a 34-year-old female is presented. In the fine-needle aspiration material, there was predominance of poorly cohesive polygonal cells with marked nuclear pleomorphism; no mitotic figures were observed. Focally, fragments of myxoid metachromatic intercellular material were also present. Histologically, the tumor was encepsulated, showing focal invasion of the capsule and tumor thrombi in the capsular vessels. The tumor was predominantly solid and myxoid, composed of cells with epithelioid features, marked anisonucleosis and a low mitotic activity. Immunohistochemically, the cells revealed positive staining for S-100 protein, vimentin, cytokeratins, glial acidic fibrillary protein and carcinoembryonic antigen; only several cell groups expressed smooth muscle actin and desmin; muscle specific actin was uniformly negative. In differential diagnosis, it was important to distinguish malignant myoepitelioma mainly from pleomorphic adenoma (mixed tumor), benign myoepithelioma, carcinoma ex pleomorphic adenoma and malignant melanoma. The criteria of malignancy in myoepithelial tumors are discussed.