骨原发性原始神经外胚层瘤临床病理学研究

目的 探讨骨原发性原始神经外胚层瘤(PNET)临床病理特点和鉴别诊断要点及组织发生。方法 观察与分析12例骨原发性PNET的临床特征、组织学特征及SP法免疫组化标记，并对其中4例进行电镜观察。结果 PNET好发于男性青少年[9～29(平均17．7)岁]的长骨。组织学特征：肿瘤由大小一致的、原始的小细胞弥漫分布，纤维性条索将其分隔成不规则片块状，可形成Homer—Wright菊形团。免疫组化标记：CD99阳性，NSE、Syn及S-100蛋白部分阳性，Vim均阳性表达。电镜下瘤细胞形态较原始，可见线粒体、发育不良及神经分泌颗粒。结论 该肿瘤是一种少见的、好发生于男性青少年、组织学形态原始的、向神经外胚层分化的、具有高度侵袭性生长的、预后不良的原发性骨肿瘤。     

伴菊形团形成的胶质神经元肿瘤5例临床病理分析

目的探讨伴菊形团形成的胶质神经元肿瘤(rosette-forming glioneuronal tumour,RGNT)的临床病理学特征。方法收集南京医科大学第一附属医院病理科2014年11月~2018年7月诊治的5例RGNT,行HE、免疫组化染色以及分子检测,并复习相关文献。结果5例均可见大小一致的神经细胞菊形团和(或)假菊形团结构,神经细胞核圆形,核仁不明显,胞质少,胞突纤细。例1、2、3可见胶质区呈毛细胞型星形细胞瘤样形态,其中例2、3见透明玻璃变的血管壁;例4部分胶质区可见少突胶质细胞瘤样形态;例4可见复杂的肾小球样血管,但细胞无明显异型性,胞核级别低,核分裂未见,无坏死。免疫表型:5例Syn在菊形团神经毡处均阳性,菊形团神经细胞Olig-2、S-100和ATRX均阳性,2例部分神经细胞NeuN阳性;5例胶质区GFAP、Olig-2和ATRX均阳性,4例S-100蛋白阳性;5例IDH1均阴性;Ki-67增殖指数<2%,其中例4球样血管增生区Ki-67增殖指数约10%。4例行PIK3CA分子检测,其中1例9号外显子(p.E545K)突变。结论RGNT是一种生长缓慢、罕见的肿瘤,有独特的组织形态,偶有恶变,目前以手术完整切除为主要治疗方法,但同时警惕潜在恶变危险,术后密切随访。     

乳腺尤因肉瘤(ES)/原始神经外胚层肿瘤(PNET)1例报道并文献复习

目的 探讨乳腺尤因肉瘤/原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNET)的病理诊断及鉴别诊断.方法 对1例乳腺尤因肉瘤/PNET进行免疫组化染色,并复习相关文献.结果镜下观察瘤组织由形态一致的小圆细胞所组成,呈实性片状或乳头状排列,局部可见菊形团样结构.免疫组化显示瘤细胞表达CD99、ER、CD56、GFAP、FLI-1、Calponin等.结论乳腺尤因肉瘤/PNET是少见的小圆细胞恶性肿瘤,CD99、FLI-1及神经内分泌标记物阳性对确诊有重要意义,ER可能在乳腺尤因肉瘤/PNET诊断中有一定的意义.     

肺纤维腺瘤4例并临床病理学观察

目的探讨肺纤维腺瘤的临床病理学特征、免疫表型、诊断及鉴别诊断。方法收集4例肺纤维腺瘤的临床病理及随访资料,行HE和免疫组化En Vision法染色,并复习相关文献。结果患者男性、女性各2例,发病年龄29~58岁(平均43岁)。4例肿物与周围肺组织境界清楚,但无明显包膜,其形态与发生在乳腺的纤维腺瘤十分相似,肿瘤组织由立方状细胞形成的腺管样结构和其间的梭形细胞形成的纤维性间质构成。免疫表型:腺样结构CK、EMA、TTF-1均阳性;梭形间质细胞CK、EMA、TTF-1均阴性,而vimentin阳性。结论肺纤维腺瘤是一种非常罕见的良性肿瘤,由于其缺乏特征性的临床表现易误诊,确诊需在HE形态学的基础上,结合免疫组化进行诊断与鉴别诊断。     

女性生殖系统原发性原始神经外胚层肿瘤5例临床病理分析

目的探讨女性生殖系统原发性原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)的临床病理学特征。方法收集5例原发于女性生殖道PNET的临床资料,应用常规病理及免疫组化En Vision两步法分析其临床病理特点,采用荧光原位杂交技术(fluorescence in situ hybridizatio,FISH)检测EWSR基因易位。结果 5例患者年龄25~79岁,其中发生于子宫2例,卵巢、外阴、阴道各1例。5例PNET肿瘤大小不等,结节状,呈圆形或者椭圆形,境界清。肿瘤实质切面灰白、灰红色,质嫩,呈鱼肉样,可见出血、坏死。镜下:肿瘤组织呈片状、条索状分布,瘤细胞体积小,大小较一致,圆形或卵圆形,细胞界限不清,胞质少,核圆形,染色质呈粗颗粒状,核分裂象多见,脉管内可见癌栓。免疫表型:CD99均阳性(5/5),vimentin均阳性(3/3),Syn阳性(4/5);其中3例检测有EWSR易位信号。随访:2例术后约1年复发死亡,1例无病生存,2例失访。结论 PNET原发于女性生殖系统罕见,在镜下形态学和免疫组化符合可以诊断,结合EWS-FLI-1融合基因可用于PNET确诊。早期明确诊断、合理的综合治疗,可使部分患者获得较好预后。由于该肿瘤恶性程度高、易早期发生转移,其长期生存率低,总体预后差。     

泌尿系统外周原始神经外胚层瘤4例临床病理分析

目的 探讨泌尿系统外周原始神经外胚层瘤(peripheral primitive neuroectodermal tumor,p PNET)的临床病理学特征、诊断、鉴别诊断及预后。方法 对4例泌尿系统pPNET行HE、免疫组化EnVision两步法染色及FISH检测,并复习相关文献。结果 4例pPNET中3例为女性,1例为男性;1例原发于膀胱,术后随访15个月未见复发;3例原发于肾脏,分别于术后3、8、13个月死亡。镜检:肿瘤细胞为形态单一的小圆细胞,可见假菊形团或Homer-Wright菊形团,核分裂象易见。免疫表型:肿瘤细胞均表达CD99、FLI-1及vimentin,部分表达Syn、NSE,Ki-67增殖指数30%~50%。FISH检测示EWSR1基因断裂。结论 泌尿系统pPNET是一种少见的高度恶性软组织肿瘤,除特征性的病理形态学改变,需结合免疫表型及细胞遗传学检测以提高诊断及鉴别诊断的准确性。     

神经母细胞瘤样上皮样神经鞘瘤

病例简介 :患者女 ,2 8岁。 5年前发现双侧上下肢多发性皮下结节 ,共 7枚。结节可活动 ,表面皮肤无色素沉着 ,患者无其他不适。其中位于右大腿的 1枚最大 ,遂取活检。病理检查 :肿瘤境界清楚、有完整包膜 ,切面实性、灰白色 ,直径 2ｃｍ。镜下观察 :肿瘤形成较大的菊形团样结构。菊形团的中央由胶原原纤维形成轴心 ,瘤细胞分布于周围(图 1)。瘤细胞有 2种形态 ,一部分为椭圆形和梭形 ,胞质较图 1　 肿瘤细胞小而圆 ,形成菊形团样结构　ＨＥ× 2 0 0少 ,围绕轴心排列成几层。而菊形团周边部分的瘤细胞则往往核深染 ,核轻度异型性 ,富于胞质 ,…     

嗅神经母细胞瘤临床病理及与其它小细胞肿瘤的鉴别诊断

目的 观察嗅神经母细胞瘤(olfactory neuroblastoma,ONB)的临床病理学特点,免疫表型及与其他小细胞肿瘤的鉴别诊断.方法 对3例ONB及其他鼻腔、鼻窦的小圆形细胞肿瘤14例,其中5例小细胞癌,1例原始神经外胚叶肿瘤,4例鼻型NK/T细胞淋巴瘤,2例胚胎性横纹肌肉瘤,1例低分化滑膜肉瘤,1例小细胞恶性黑色素瘤进行形态学和免疫组化观察.结果 3例ONB形态学特征:瘤细胞大小较一致,高密度小细胞,少许纤维状胞质,核圆形深染,瘤细胞排列成分叶状,或弥漫状分布,间质为丰富的纤维血管,可见Homer-Wright菊形团、Flexner菊形团和神经丝分化.2例为低级别肿瘤,1例为高级别肿瘤.免疫表型:3例NSE弥漫阳性表达,S-100蛋白特征性表达肿瘤周边的支持细胞为主要特征,2例灶性NF、Syn、CgA表达,Ki-67瘤细胞表达率高,而desmin、myogenin、HMB45、bcl-2、CK19、CD99、CD45、CD45RO均阴性.随访3例,1例2.5年无瘤生存,1例2年内复发,1例9年后多处转移,死亡.结论 ONB是一种少见的特殊类型的恶性神经外胚叶肿瘤,诊断时应与其他的小圆形细胞肿瘤相鉴别.     

透明细胞肉瘤临床病理分析

目的探讨透明细胞肉瘤（clear cell sarcoma,CCS）的临床病理特点及诊断与鉴别诊断。方法对9例CCS进行临床病理分析,并行组织学、免疫组化染色和电镜观察。结果9例CCS患者年龄19～62岁,平均38．6岁;肿瘤多数位于肢体末端（6／9）。组织学观察：肿瘤由被纤维组织分隔成巢或束状的胞质透亮或淡伊红染的上皮样细胞和梭形细胞构成,核圆形或卵圆形,较空淡,可见明显核仁。1例见少数多核巨细胞,1例胞质内含色素颗粒,2例间质黏液样变性,1例局部侵及表皮。术后随访6—24个月,6例中2例局部复发伴转移。免疫组化观察：9例CCS均表达vimentin和HMB-45,7／9例S-100蛋白阳性,5／9例NSE阳性,4／9例melan-A阳性,1／9例Syn阳性。电镜观察：胞质内可见不同时期的黑色素小体。结论CCS是好发年轻人肢体末端的软组织肉瘤,预后不良,属高度恶性。诊断应结合临床与病理形态,免疫组化在诊断和鉴别诊断中起重要作用。     

肝圆韧带透明细胞肌黑色素细胞瘤

目的：探讨肝圆韧带透明细胞肌黑色素瘤的临床病理特征。方法：通过HE、组化、免疫组化及电镜观察1例透明细胞肌黑色素细胞瘤。结果：女性22岁,发生于肝圆韧带,肿瘤由中等长梭形及上皮样梭形细胞组成,胞质透明或淡染伊红、胞核圆或卵圆形居中或偏好,明显小红核仁,核分裂象罕见,肿瘤内富有明显玻璃样变厚壁血管,呈分支状、条索状或团块状广泛分布,将瘤细胞分隔成束状或巢状结构,组化染色显示胞质富含糖原颗粒,黑色素染色阴性。免疫表型：HMB45强阳性,SMA及actin阳性,S－100蛋白部分阳性。超微结构：瘤细胞质内可见Ⅱ-Ⅲ期黑色素小体。结论：该例为具有独特的、以梭形透明细胞为主的肌样黑色素细胞肿瘤,属于血管周细胞肿瘤家族中的一新成员。     

Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections.

The differential diagnosis of small round cell tumors is exhaustive and requires ancillary studies. Relatively recently, fluorescence in situ hybridization (FISH) using probes for specific gene rearrangements has gained wide acceptance. This technique is particularly useful in the differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) and desmoplastic small round-cell tumor (DSRCT). In ES/PNET, the EWS gene is juxtaposed to the FLI-1 gene in 85% of cases and to the ERG gene in another 7% of cases; the EWS gene is juxtaposed to the WTI gene in DSRCT. Documentation of the EWS gene rearrangements in EWS/PNET has previously been demonstrated in frozen tissue. We report 2 unusual cases of EWS/PNET diagnosed in abdominal tumors in adults. Although the immunohistochemical results supported a diagnosis of ES/PNET, 1 case morphologically resembled DSRCT. The diagnosis in these 2 cases was confirmed by the FISH demonstration of EWS/FLI-1 gene fusion in paraffin-embedded tissue. Thus, the usefulness of FISH demonstration of an EWS gene rearrangement with these specific probes in such unusual cases is supported and is demonstrated in paraffin-embedded tissue.     

类似卵巢性索肿瘤的子宫肿瘤6例临床病理分析

目的探讨类似卵巢性索肿瘤的子宫肿瘤(uterine tumor resembling ovarian sex cord tumor,UTROSCT)的临床病理学特征、分子遗传学改变及其鉴别诊断。方法回顾性分析6例UTROSCT的临床病理学特征、免疫表型等。采用FISH检测JAZF1基因及NCOA2基因易位情况,并复习相关文献。结果6例患者年龄43~58岁,平均51.3岁。肿瘤最大径2.0~11.0 cm,切面多为灰白、灰黄色。镜检:肿瘤形态包括相互吻合的条索、小梁和小管样、网状和实性结构;肿瘤间质稀少伴玻璃样变,可见数量不等的动静脉血管,间质内均无明显螺旋小动脉。肿瘤细胞呈卵圆形,可见明显核仁,偶见核沟;核分裂象少见。免疫表型:肿瘤具有多向分化的免疫表型。6例肿瘤均表达至少2种性索分化的标记(CD99、CD56、Calretinin、WT-1、α-inhibin),不同程度表达平滑肌标记(desmin、H-Caldesmon、SMA);5例表达vimentin,4例表达上皮标记(CKpan),3例表达子宫内膜间质标记(CD10)。6例均不表达S-100蛋白、HMB-45等标志物。Ki-67增殖指数1%~30%。4例行FISH检测,均未发现JAZF1及NCOA2基因相关易位。5例患者获得随访,随访时间5~61个月,均无瘤生存。结论UTROSCT是一种罕见的具有多种免疫表型的子宫肿瘤,确诊主要依靠病理形态学检查及免疫组化标记,必要时行分子病理检查,治疗以手术切除为主,预后较好。     

Cytokeratin-positive meningeal peripheral PNET/Ewing's sarcoma of the cervical spinal cord: diagnostic value of genetic analysis

Peripheral primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES) of the central nervous system is extremely rare and should be differentiated from central PNET and other small blue round cell tumors. We describe a case of a meningeal peripheral PNET/ES of the spinal cord in an 11-year-old boy. Immunohistochemically, the small blue round cell tumor showed expression of epithelial markers and of CD99, thus posing an important differential diagnostic problem with a poorly differentiated synovial sarcoma. Fluorescence in situ hybridization revealed rearrangement of the EWS gene, as seen in peripheral PNET/ES. Peripheral PNET/ES does occur in the central nervous system, but its diagnosis can be extremely difficult on morphologic and immunohistochemical grounds alone. Genetic analysis plays a key role in its distinction from other small blue round cell tumors.     

Mixed epithelial and stromal tumor of the kidney: A retrospective clinicopathological evaluation

ObjectiveMixed epithelial and stromal tumor of the kidney (MESTK) is a rare renal tumor for which there is limited clinical case experience. The aim of this study was to investigate the clinicopathological features, imaging manifestations, immunohistochemical phenotype, diagnosis, and differential diagnosis of MESTK.MethodsWe systematically evaluated the clinicopathological characteristics of 13 patients diagnosed with MESTK from 2016 to 2022.ResultsThere were 12 female cases and 1 male case, and their age ranged from 24 to 62 (mean age 47 years). The tumor was located in the left kidney in 11 patients. Seven of these patients were located in the upper pole of the left kidney. The mean diameter of the tumor was 4.6 cm (range 1.3–8.6 cm), and the main body of the tumor was mainly located in the medulla or medulla. In 7 cases, the tumors were clearly outlined, 4 cases had clear but irregular borders, and in 3 cases, the masses broke through the renal capsule but did not invade the surrounding organs. All tumors had varying proportions of cystic and solid components. In most tumors, the epithelial cell component was predominant. Among the epithelial components, we observed a predominance of large cysts. Small cysts and tubular structures were also seen; the least common were papillary structures. The most common types of lining epithelium were flat and cuboidal. The cell cytoplasm was predominantly eosinophilic with hyaline degeneration. The tumor cells were composed of different proportions of sparse hypocellular areas and dense hypercellular areas between these cells. In most cases, the cellular stroma had an ovarian-like appearance. It is characterized histologically by mature adipocytes, thick-walled blood vessels, and chronic inflammatory cells. Calcification and collagenization were seen in a few cases. In all female patients, estrogen and progesterone receptors in the interstitial component were almost always positively expressed. In male patients, all interstitial components showed locally positive expression of androgens.ConclusionMESTK had unique characteristics with complex and variable lesion shapes. There was a high degree of overlap with cystic kidney cancer, and the rate of missed and misdiagnosis was extremely high. The diagnosis could not be confirmed by preoperative imaging. The final diagnosis depended on pathomorphology.     

混合性嗜铬细胞瘤临床病理学特征分析

目的探讨混合性嗜铬细胞瘤(composite pheochromocytoma,CP)临床病理特征、免疫表型、诊断及鉴别诊断。方法收集2011年1月至2019年1月浙江省人民医院收治的5例CP,对5例CP的临床、影像学、组织学、免疫组织化学特征及预后进行观察,探讨其临床病理特征及鉴别诊断。结果CP患者中男性4例,女性1例,男女发病比例为4∶1,发病年龄52~68岁(平均年龄59岁,中位年龄54岁),肿瘤最大径3~4 cm(平均最大径3.6 cm,中位最大径3.5 cm);临床表现多为无明显症状的肾上腺肿块;典型的形态为两种成分组成,一种为瘤细胞呈不规则巢状排列,瘤细胞胞质细颗粒状、嗜碱性或双嗜性,核分裂象罕见;另一种为由不规则交叉束状排列的施万细胞及散在分布的神经节细胞组成;免疫组织化学示嗜铬细胞瘤弥漫表达PHOX2B(5/5)、突触素(5/5)、嗜铬粒素A(CgA,5/5),支持细胞S-100蛋白阳性(5/5),节细胞神经瘤弥漫表达S-100蛋白(5/5)、神经丝蛋白(5/5),其内神经节细胞PHOX2B、突触素、CgA弱阳性;5例均接受手术切除,预后良好,随访均无复发。结论CP较少见,临床表现不具有特异性,其具有独特的组织学特征,仔细的观察肿瘤的完整组织结构及免疫组织化学检测PHOX2B、CgA、突触素、S-100蛋白有助于其诊断和鉴别诊断。     

Histological heterogeneity of Ewing’s sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support

Ewing’s sarcoma (ES)/peripheral neuroectodermal tumor (PNET) are malignant neoplasms affecting children and young adults. We performed a study to typify the histological diversity and evaluate antibodies that may offer diagnostic/prognostic support. In total, 415 cases of genetically confirmed paraffin-embedded ES/PNET were analyzed on whole sections and in tissue microarrays. This study confirms the structural heterogeneity of ES/PNET, distinguishing three major subtypes: conventional ES (280 cases); PNET (53 cases); and atypical ES/PNET (80), including large cells, vascular-like patterns, spindle pattern, and adamantinoma-like configuration. All cases presented positivity for at least three of the four tested antibodies (CD99, FLI1, HNK1, and CAV1). CAV1 appeared as a diagnostic immunomarker of ES/PNET being positive in CD99-negative cases. Hence, the immunohistochemical analysis confirmed the diagnostic value of all four antibodies, which together cover more than 99% of the tumors, independently of the histological variety. The univariate analysis for survival revealed atypical ES as the only histological parameter apparently associated with less favorable clinical outcome, particularly in the subgroup of patients treated with surgery. In conclusion, the diagnosis of atypical ES is a challenge for the pathologist and needs support from molecular techniques to perform an optimal differential diagnosis with other small round cell tumors.     

巨细胞纤维母细胞瘤的临床与病理学观察

目的：研究巨细胞纤维母细胞瘤的临床病理学特点和免疫组织化学表型,探讨其鉴别诊断及组织学起源。方法：采用光镜观察结合免疫组织化学LSAB法标记对7例巨细胞纤维母细胞瘤进行分析。结果：7例患者中6例为儿童,1例为35岁成年人。男性5例,女性2例。临床上主要表现为躯干和四肢皮下缓慢增大的无痛性结节,平均直径2．9cm。镜下显示,肿瘤境界不清,主要位于真皮层。瘤细胞主要由轻至中度异型的梭形细胞组成,多呈疏松的束状或波浪状排列,间质呈纤维黏液样,部分区域呈致密胶原化。本病的特征性形态表现肿瘤内含有一些不规则分布的裂隙样或扩张窦样的假脉管性腔隙,其腔隙面内衬一层不连续的梭形细胞和核深染多核巨细胞,两种细胞在形态上有移行。免疫组织化学标记显示梭形细胞和多核巨细胞均表达波形蛋白和CD34.5 附有随访资料,其中2例术后复发。结论：（1）巨细胞纤维母细胞瘤是一种好发于儿童的中间性纤维母细胞性肿瘤,较易局部复发,掌握其独特的临床病理学特征对避免误诊为一些具有相似形态的病变具有重要意义;（2）巨细胞纤维母细胞瘤与好发于成人的隆突性皮纤维肉瘤的在临床表现、免疫组织化学、细胞及分子遗传学上均极为相似,在组织上也可共存,提示两者在组织学发生了关系密切,可能同属CD34阳性树突状纤维母细胞肿瘤一族。     

Diagnostic utility of MIC-2 immunocytochemical staining in the differential diagnosis of small blue cell tumors

Ewing's sarcoma (ES) and peripheral neuroectodermal tumor (PNET) are considered in the differential diagnosis of small round blue cell tumors of infancy and childhood which includes neuroblastoma, rhabdomyosarcoma and malignant lymphoma. Fine-needle aspiration diagnosis of these neoplasms can be particularly difficult when the neoplasms are composed of poorly differentiated cells or fail to produce a stroma. MIC-2 is a highly sensitive and specific marker for the PNET/ES group of neoplasms and has been studied extensively in surgical pathology. Other small blue cell neoplasms including rhabdomyosarcoma, blastemal Wilm's tumor, and lymphoblastic lymphoma have also shown positivity, but the staining reactions are usually weak and focal. The utility of this marker in the differential of small blue cell neoplasms in cytologic material has not been examined. Twenty cases of small blue cell neoplasms obtained by fine-needle aspiration (FNA) were studied. MIC-2 antibody was applied retrospectively to formalin-fixed cell block material and destained alcohol-fixed and air-dried cytologic preparations. These cases include primitive neuroectodermal tumor (five cases), Ewing's sarcoma (two cases), neuroblastoma (four cases), Wilms's tumor (four cases), lymphoblastic lymphoma (two cases), and small-cell carcinoma (three cases). The cases were judged positive when the majority of the cells showed cytoplasmic staining. Diffuse cytoplasmic staining was observed in all seven cases of PNET/ES. Staining could be seen on the destained air-dried smears (three cases), fixed smears (two cases), or the cell block material (two cases). None of the other 13 small blue cell neoplasms showed positive staining. We conclude that MIC-2 is a sensitive and specific marker for the PNET/ES group of neoplasms in specimens from formalin-fixed cell block, air-dried, and alcohol-fixed cytologic material and is useful in the differential diagnosis of small blue cell tumors. Diagn. Cytopathol. 1998;19:410–416. © 1998 Wiley-Liss, Inc.     

子宫和卵巢腺瘤样瘤的临床病理分析

目的 探讨女性生殖系统腺瘤样瘤的发生、免疫组织化学表达的特征及鉴别诊断。方法 对24例子宫和卵巢腺瘤样瘤进行临床病理及免疫组织化学观察。结果 24例患者中腺瘤样瘤发生于子宫者21例,卵巢者2例,子宫与卵巢同时发生者1例,分别占本院同期子宫及卵巢肿瘤及瘤样病变的0.34%和0.06%。免疫组织化学染色显示：波形蛋白及细胞蛋白（AE1／AE3）均为阳性,呈双相表达;第八因子相关抗原（FⅧRAg)均为阴性;S－100蛋白20例阳性（83.3%),上皮膜抗原（EMA）4例阳性（16.7%);其中10例行calretinin蛋白染色,均为阳性表达。结论 女性生殖系统腺瘤样瘤为间皮起源,子宫为最常见部位。免疫组织化学染色结果可作为诊断及鉴别诊断的重要参考依据。其生物学行为为良性,预后良好。     

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.