血管周上皮样细胞肿瘤免疫表型分析

目的 研究血管周上皮样细胞肿瘤(PEComa)的免疫表型和分子遗传学改变及其与临床病理特征的关系.方法 收集25例PEComa患者的存档蜡块、病理及临床资料,采用免疫组织化学(SP法或EnVision法)检测相关免疫标志物,同时以多种肿瘤进行对照.运用荧光原位杂交(FISH)方法检测25例PEComa中TFE3基因的易位及扩增情况.结果 25例患者年龄21 ～61岁(平均43岁),男女比例为1∶1.3.22例为肝脏和肾脏的血管平滑肌脂肪瘤(AML),由成熟脂肪组织、梭形或上皮样平滑肌细胞和特征性的厚壁血管组成;3例为肝肾外PEComa,形态与经典AML有明显区别,肿瘤由单行性上皮样或梭形细胞构成,呈片状、巢状排列.免疫组织化学结果显示:25例PEComa组织蛋白酶K(cathepsin K)均呈强阳性(100％,25/25),HMB 45、Melan A和平滑肌肌动蛋白(SMA)的阳性率分别为80％ (20/25)、88％ (22/25)和88％(22/25),阳性瘤细胞占所有瘤细胞的百分比平均值:cathepsin K为90％、HMB 45为36％、Melan A为41％、SMA为35％.TFE3在肝肾AML中全部阴性(22/22),而在肝肾外PEComa中均为阳性(3/3).经FISH证实25例PEComa均不存在TFE3基因融合或扩增.结论 肝肾外PEComa的组织学形态与经典AML有明显区别,其发病与TFE3蛋白高表达关系密切,可能为独立的PEComa分子亚型.cathepsin K在PEComa中阳性率高,敏感性优于HMB 45、Melan A和SMA,可用于PEComa与其他常见的多种肿瘤的鉴别诊断.     

肝脏单形性上皮样血管平滑肌脂肪瘤

目的：探讨肝脏单形性上皮样血管平滑肌脂肪瘤的临床病理学特征及诊断、鉴别诊断要点。方法：对1例单形性上皮样血管平滑肌脂肪瘤进行临床病理学分析及免疫组织化学研究。结果：肝脏单形性上皮样血管平滑肌脂肪瘤临床多无症状,光镜下单形性上皮样血管平滑肌脂肪瘤由形态多样的上皮样细胞构成,胞质透明或嗜酸,无脂肪组织及异常血管;免疫表型;HMB45阳性,SMA及vimentin部分阳性,desmin少数阳性,S－100蛋白弱阳性,cytokeratin及AFP阴性,CD34血管内皮细胞阳性。结论：肝脏单形性上皮样血管平滑肌脂肪瘤是极为罕见的间叶性肿瘤,组织起源至今不明,其诊断及鉴别诊断主要依靠病理组织学及免疫组织化学。     

网膜和肠系膜胃肠道外间质瘤的临床病理研究

目的 研究网膜和肠系膜胃肠道外间质瘤(EGIST)临床病理和免疫组织化学标记特点,并探讨其组织来源、预后评价及与胃肠道间质瘤(GIST)的关系。方法 运用形态学和免疫组织化学(CD117、CD34等)研究19例网膜和肠系膜原诊断为平滑肌肿瘤、许旺瘤的间叶性肿瘤。结果 共诊断14例EGIST,其中网膜6例,肠系膜8例。肿瘤大小3．5～29．0cm(平均12．4cm)。梭形细胞为主型9例,上皮样细胞为主型2例,混合型3例。免疫组织化学阳性表达结果：CD117(14／14)、CD34(8／14)、α-平滑肌肌动蛋白(6／14)。结蛋白、S-100蛋白均阴性。随访结果：6例网膜EGIST均无瘤生存;7例肠系膜EGIST3例死于肿瘤,1例带瘤生存,3例无瘤生存。结论 EGIST与GIST为同一性质肿瘤,可能共同起源于多分化潜能的间叶干细胞或肿瘤向卡哈尔间质细胞分化。EGIST有独特的行为谱,预后评价不能完全套用GIST的评价指标。     

胃肠、泌尿、会阴部间质瘤临床病理及免疫组织化学分析

目的 探讨胃肠道间质瘤(GIST)与胃肠道外GIST型间质瘤的组织学起源与病理特征。方法 对46例胃肠道及13例泌尿道、会阴部原诊断平滑肌瘤、平滑肌肉瘤、许旺瘤的病例作回顾性研究,观察其病理特点,应用免疫组织化学方法观察4种抗体(CD117、CD34、平滑肌肌动蛋白、S—100)的表达,对发生于不同部位的间质瘤进行对比分析。结果 45例为GIST组,CD117阳性表达率为93．3％,CD34阳性率88．9％;12例为胃肠道外GIST型间质瘤组,CDll7阳性表达率为83．3％,CD34阳性率75．0％;2例(其中1例为胃肠道)平滑肌瘤组,CDll7和CD34均为阴性,平滑肌肌动蛋白瘤细胞呈弥漫性强阳性表达。结论 CDll7和CD34标记阳性是确诊间质瘤最具有诊断价值的依据。推测GIST和胃肠道外GIST型间质瘤均系起源于一种非定向分化的、原始间充质干细胞。     

腹膜后恶性血管周上皮样细胞肿瘤的临床病理分析

目的:探讨腹膜后血管周上皮样细胞肿瘤(perivascular epithelioid cell neoplasms,PEComas)的临床和病理学特点,提高对PEComas的认识和诊断水平。方法:对1例腹膜后恶性PEComas进行临床病理分析及免疫组织化学研究,并复习相关文献。结果:肿瘤组织主要由上皮样细胞构成,胞质丰富、透明或嗜酸性颗粒状,间质富于血管。免疫组织化学显示:肿瘤细胞平滑肌肌动蛋白、HMB45,melan A及Desmin阳性,上皮膜抗原和vimentin局灶阳性,CgA,Syn,S-100,CD117,CD34,细胞角蛋白,calretinin及inhibin均阴性。结论:腹膜后PEComas是一种非常罕见的肿瘤,具有独特的组织学和免疫组织化学特征,应与腹膜后其他上皮样细胞肿瘤进行鉴别。     

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。     

胃肠道间质瘤临床病理及免疫组织化学特征

目的 探讨胃肠道间质瘤的免疫组织化学特征，为其诊断及鉴别诊断和预后提供依据。方法 对消化道内169例间叶源性肿瘤进行免疫组织化学标记和形态学观察，确诊113例胃肠道间质瘤。结果 肿瘤多见于胃，临床常见首发症状为消化道出血及腹部包块。瘤细胞主要有梭形细胞及上皮样细胞两种形态，梭形细胞型70例，上皮样细胞型10例，混合细胞型33例。相对良性33例，交界性26例，恶性54例。免疫表型：CD117阳性112例，CD34阳性102例，阳性率分别为99．1％及90．2％，且呈弥漫强阳性表达。结论 胃肠道间质瘤是消化道最常见间叶源性肿瘤，以胃内多见；主要有2种细胞形态和3种组合形式；确诊需要依靠CD117、CD34等免疫标记物配合。     

74例胃肠道间质瘤临床病理与生物学行为评价

目的　探讨在胃肠道间质瘤(GIST)的病理诊断和预后分析上采用一种简单实用且重复性好的病理学“标准”,以利于GIST的日常病理诊断和生物学行为评价及指导治疗,并对Fletcher等推荐的GIST生物学行为评价表进行评估。方法　85例消化道间叶组织肿瘤,复习其病理形态学并应用CD117、CD34、平滑肌肌动蛋白(SMA)、结蛋白、S 100等进行免疫组织化学标记,结合 31例随访资料进行分析。结果　85例消化道间叶组织肿瘤中,GIST74例,平滑肌瘤和交界性平滑肌瘤 8例(食管),平滑肌肉瘤 1例(直肠 ),神经鞘瘤 1例 (胃 ),恶性纤维组织细胞瘤 1例 (肠系膜 )。74例GIST中,发生在胃和小肠的分别为 34例和 30例,占 86. 5%,食管 3例,胃肠道外(肠系膜、网膜、后腹膜)7例。年龄 23~80岁,平均 52 5岁, 40岁以上者占 85%,男性 45例,女性 29例。镜下观察:梭型细胞型 48例,上皮样细胞型 10例,混合细胞型 16例。瘤细胞呈长、短梭形和圆形,胞质丰富弱嗜酸性,排列呈旋涡状、栅栏状或弥漫巢状。免疫组织化学: 85例消化道间叶组织肿瘤波形蛋白均阳性,其中 74例表达CD117,诊断为GIST,表达形式有弥漫胞膜 /胞质强阳性、散在阳性、胞质点状着色等,其中 54例同时表达CD34 (阳性率 72. 9% ), 25例表达SMA, 5例表达结蛋白, 5例表达S 100蛋白。在 85例     

原发性骨外骨肉瘤十例临床病理学研究

目的探讨原发性骨外骨肉瘤(extraskeletal osteosarcoma,ESOS)的临床病理、免疫组织化学及分子病理学特征。方法收集2003年1月至2019年1月福建省立医院诊治的10例ESOS,进行HE、免疫组织化学染色及分子病理学检测,并电话随访及复习相关文献。结果3例女性和7例男性,年龄36~85岁(平均年龄60岁),肿块大小5.5~17.5 cm(平均11.0 cm)。低倍镜下,肿瘤呈结节状、片状、分叶状,肿瘤由梭形细胞、肿瘤性骨样组织、软骨样组织构成,三者比例多少不等,并相互移行,其中见异型性梭形细胞直接产生骨样组织。免疫表型:肿瘤细胞呈SATB2部分阳性(9/9),α-平滑肌肌动蛋白(4/10)和上皮细胞膜抗原(1/10)灶性阳性,Ki-67阳性指数10%~50%,结蛋白、CD68、S-100蛋白、SOX10、HMB45、CD117、DOG1、CD34、广谱细胞角蛋白(CKpan)、GATA3及PAX8均阴性。分子病理检测:未检测到MDM2/CDK4基因扩增信号(0/6);未见SSX18基因分离信号(0/5);未检测到C-KIT和PDGFR-α突变信号(0/3)。结论ESOS属于骨外成骨性肿瘤,诊断需临床、影像、病理学相结合,必要时免疫组织化学及分子病理检测辅助诊断。     

滤泡性树突状细胞肉瘤临床病理观察

目的探讨滤泡性树突状细胞肉瘤的临床病理特点及免疫表型,提高对该肿瘤的认识和诊断水平。方法通过光镜、电镜和免疫组织化学染色[EnVision法,所选用抗体为：CK（AE1／AE3）、S-100蛋白、CD1a、CD21、CD23、CD35、CD34、CD68、波形蛋白、结蛋白、HMB45、p53]观察并结合临床资料对5例滤泡性树突状细胞肉瘤进行临床病理分析。5例均获随访。结果5例患者中男3例,女2例,平均年龄37岁。肿瘤均位于头颈部淋巴结。镜下观察：肿瘤组织呈片巢状、束状或旋涡状排列,瘤细胞卵圆形或梭形,胞质丰富淡嗜酸性;核卵圆形或胖梭形,趋向不规则成簇分布,散见多核巨细胞;核染色质稀疏,核仁小而清楚;核分裂象数目不等,有时显示明显的核异型。瘤细胞CD21、CD23、CD35阳性,少数瘤细胞CD68、S-100蛋白阳性,CD1a、CD34、HMB45、CK均阴性。电镜下瘤细胞有长而明显的绒毛状胞质突起及特征性的桥粒样连接,未见Birbeek颗粒。随访5～52个月（平均26个月）,无复发或再复发及转移。结论滤泡性树突状细胞肉瘤是一种少见的恶性肿瘤,预后不确定。正确诊断需要病理组织形态、电镜及免疫组织化学相结合,并应与朗格汉斯细胞肉瘤、指突状树突状细胞肉瘤、恶性纤维组织细胞瘤、黑色素瘤、梭形细胞癌等相鉴别。     

腹内胃肠道外间质瘤临床病理、免疫组织化学及分子遗传学研究

目的探讨腹内胃肠道外间质瘤(extra-gastrointestinal stromal tumors,EGIST)临床病理、免疫组织化学、分子遗传学特点及鉴别诊断。方法用CD117、CD34为主的一组抗体对消化道外腹腔软组织原诊断为平滑肌瘤、平滑肌母细胞瘤及平滑肌肉瘤等病例进行研究,获得9例EGLST,其中5例检测了c-kit基因11号外显子序列。结果患者中男性5例,女性4例,年龄38～72岁,平均61．7岁,其中肠系膜4例,网膜2例,腹膜后2例,1例位于脾门,肿瘤直径5～23cm,平均12．9cm。梭形细胞为主型7例,上皮型1例,混合型1例。此组抗体表达分别为CDll7(8／9)、CD34(5／9)、平滑肌肌动蛋白(d—SMA,3／9)、肌特异性肌动蛋白(MSA,4／9)、结蛋白(0／9)、s-100蛋白(1／9)、蛋白基因产物9．5(PGP9．5,1／9)。2例有c-kit基因11号外显子杂合性突变。交界性2例,分别存活8年和11年,恶性7例,1例无瘤生存4年,1例1年后死于肝转移,1例术后3年及4年两次复发,2例失访,2例随访中。结论消化道外腹腔软组织及腹膜后亦可发生符合GIST形态学、免疫表型及分子生物学特征的原发性间质瘤,生物学行为以交界性及恶性多见,肿瘤性坏死、核分裂象≥5／50HPF及细胞明显异型性对判断恶性有重要参考价值。需与相同部位的平滑肌肉瘤、恶性神经鞘膜瘤等鉴别。     

Angiomyolipoma mimicking true lipoma of the liver: report of two cases

Hepatic angiomyolipoma (AML) is very rare and only about 80 cases have been reported. The tumor is fundamentally heterogeneously composed of the three tissue components of blood vessels, smooth muscle cells (SMC), and fat cells. Two cases of hepatic AML are reported here, both of which are histologically composed predominantly of a fat cell element and resembled true lipoma (lipomatous AML). However, careful examination of both tumors revealed the presence of a small amount of epithelioid SMC, especially around blood vessels. Immunohistochemical study using monoclonal antibody for melanoma (HMB-45) clearly revealed a small amount of HMB-45-positive SMC around the blood vessels and scattered in the diffuse fat cell growth in both tumors. Since no liver tissue components or primary liver tumors are reactive with HMB-45 except AML cells, the presence of HMB-45-positive cells within the tumor clearly established the diagnosis of hepatic AML. Any fatty tumor or focal fatty lesion of the liver that superficially resemble true lipomas should be tested for the presence of HMB-45-positive SMC in the tumor to differentiate it from AML.     

Follicular dendritic cell sarcoma mimicking giant cell carcinoma of the pancreas

Extranodal follicular dendritic cell (FDC) tumors are rare. Recognition of the morphological spectrum of FDC tumors is important to clinical diagnosis. Herein is presented a case of pancreatic FDC sarcoma with unusual clinicopathological features. A 64-year-old male patient presented with weight loss, poor appetite, abdominal fullness, mild anemia and mild peripheral eosinophilia. Histologically, the tumor was composed of both epithelioid and spindle cells with abundant intracytoplasmic hyaline globules. These tumor cells were positive for CD21, CD23, CD35, S-100 protein, fascin and clusterin. Both epithelioid and spindle tumor cells independently colonized the liver and formed two tumor nodules 18 months after the initial resection. Notably, the two hepatic metastases additionally acquired patchy expression of human leukocyte antigen-DR. The epithelioid FDC in one of the hepatic lesions transformed into numerous bizarre giant cells, which could easily be confused with a metastatic giant cell carcinoma from the pancreas. FDC tumor should therefore be included in the differential diagnoses when dealing with a giant cell tumor.     

Immunohistochemical study of hepatic angiomyolipoma

An immunohistochemical study was performed on nine hepatic angiomyolipomas (AML) found in eight patients. Histologically, the tumors were fundamentally composed of the three heterogeneous tissue components of blood vessels, smooth muscle cells (SMC), and fat cells, although the proportions and distributions were quite variable from tumor to tumor and from area to area in the same tumor. Additionally, cellular pleomorphism and atypia with occasional bizarre giant cells were found in the SMC component. This histologic feature might lead to a mistaken diagnosis of malignant neoplasm, and pathologists should therefore be aware of the broad histologic spectrum of hepatic AML. However, the immunostaining patterns were basically the same in all nine tumors. All tumor components were negative for epithelial membrane antigen (EMA) and for cytokeratin. The spindle-shaped SMC component of the tumor was occasionally positive for vimentin, desmin and alpha-smooth muscle actin, whereas epithelioid SMC were negative for all three. Both the epithelioid and spindle-shaped SMC were occasionally positive for S-100 and neuron-specific enolase. All types of SMC in the tumor, whether spindle, epithelioid, intermediate or pleomorphic SMC, were strongly positive for HMB-45, a melanoma-specific monoclonal antibody. Fat cells were occasionally positive for S-100. Endothelial cells were positive for factor VIII-associated antigen. Among hepatic tumors HMB-45 reactivity is, so far as we know, found exclusively in the SMC of AML, and the HMB-45 reactivity of a hepatic tumor is thus clearly an important piece of information in the diagnosis of AML.     

Sclerosing variant of epithelioid angiomyolipoma

Presented herein are two unusual epithelioid angiomyolipomas (AML) displaying prominent stromal sclerosis. Both patients were middle-aged women without a clinical history of tuberous sclerosis. One patient (case 1) had a 2 cm lesion arising in the renal cortex, and another (case 2) had a pararenal retroperitoneal tumor measuring 13 cm. Both tumors were composed of sheets or nests of polygonal epithelioid or short spindle cells having uniform round to oval nuclei and eosinophilic cytoplasm with cords of hyalinized sclerotic stroma between them. The tumor in case 2 had small areas of mature-looking fat cells. Immunohistochemically, epithelioid tumor cells were diffusely positive for actins and desmin in both cases, and melanoma antigen recognized by T cells (MART)-1 was positive in patient 2. Scattered HMB-45-immunoreactive cells were identified in the sclerotic cords of both tumors, but epithelioid tumor cells were essentially negative for HMB-45. The characteristic clinicopathological and immunohistochemical features of the present cases are analogous to a subset of epithelioid AML or sclerosing perivascular epithelioid cell tumors previously reported.     

MDS/AML-associated cytogenetic abnormalities in multiple myeloma and monoclonal gammopathy of undetermined significance: evidence for frequent de novo occurrence and multipotent stem cell involvement of del(20q)

Multiple myeloma (MM) and monoclonal gammopathy of undetermined significance (MGUS) are characterized cytogenetically by 14q32 rearrangements, -13/13q-, and various trisomies. Occasionally, karyotypic patterns characteristic of myelodysplastic syndrome (MDS)/acute myeloid leukemia (AML) occur in MM, often signifying therapy-related (t)-MDS/t-AML. Comparison of cytogenetic features in all published MMs (n = 993) and t-MDS/t-AML post-MM (n = 117) revealed significant differences in complexity and ploidy levels and in most genomic changes. Thus, these features often can be used to distinguish between MM and t-MDS/t-AML. Rarely, myeloid-associated aberrations are detected in MM without any signs of MDS/AML. To characterize such abnormalities in MM/MGUS, we ascertained all 122 MM and 26 MGUS/smoldering MM (SMM) cases analyzed in our department. Sixty-six (54%) MMs and 8 (31%) MGUS/SMMs were karyotypically abnormal, of which 6 (9%) MMs and 3 (38%) MGUS/SMMs displayed myeloid abnormalities, that is, +8 (1 case) and 20q- (8 cases) as the sole anomalies, without any evidence of MDS/AML. One patient developed AML, whereas no MDS/AML occurred in the remaining 8 patients. In one MGUS with del(20q), fluorescence in situ hybridization analyses revealed its presence in CD34+CD38- (hematopoietic stem cells), CD34+CD38+ (progenitors), CD19+ (B cells), and CD15+ (myeloid cells). The present data indicate that 20q- occurs in 10% of karyotypically abnormal MM/MGUS cases and that it might arise at a multipotent progenitor/stem cell level.     

Spectrum of cytopathologic features of epithelioid sarcoma in a series of 7 uncommon cases with immunohistochemical results,including loss of INI1/SMARCB1 in two test cases

Diagnosis of an epithelioid sarcoma (ES) is challenging on fine needle aspiration cytology (FNAC) smears. There are few documented series describing cytopathologic features and immunostaining results of ESs. The present study describes cytopathologic features of seven cases of ES. All seven tumors occurred in males within age‐range of 22–61 years; in sites, such as forearm (n = 3), hand (n = 2), thigh (n = 1), and inguinal region (n = 1). FNAC was performed for metastatic lesions (n = 5), recurrent lesions (n = 4), as well as for a primary diagnosis (n = 1). FNAC smears in most cases were moderate to hypercellular, composed of polygonal cells(seven cases) and spindle cells(three cases), arranged in loosely cohesive groups, non‐overlapping clusters, and scattered singly, containing moderate to abundant cytoplasm, defined cell borders, vesicular nuclei, and discernible nucleoli. Variable cytopathologic features identified in certain cases were “rhabdoid‐like” intracytoplasmic inclusions (n = 5), giant cells (n = 3), and interspersed scanty, metachromatic stroma (n = 4). Histopathologic examination revealed two cases of conventional‐type ES, three of proximal/large cell‐type ES, and two cases of mixed‐type ES, displaying features of conventional and proximal subtypes. By immunohistochemistry (IHC), tumor cells were positive for cytokeratin (CK)(4/5), epithelial membrane antigen (EMA) (6/6), panCK (1/1), vimentin (3/3), and CD34 (7/7). Tumor cells were completely negative for INI1/SMARCB1 (0/2) and CD31 (0/5). In our settings, FNAC was mostly performed in recurrent and/or metastatic cases of ES, and rarely for a primary diagnosis of ES. Important cytopathologic features of ESs include loosely cohesive, non‐overlapping clusters of polygonal cells with variable “rhabdoid‐like” and spindle cells. Optimal diagnostic IHC markers in such cases include CK, EMA, AE1AE3, CD34, and INI1/SMARCB1. Clinical correlation is imperative in all cases. Diagn. Cytopathol. 2016;44:636–642. © 2016 Wiley Periodicals, Inc.     

腹腔上皮样炎性肌纤维母细胞肉瘤临床病理分析并文献复习

目的：通过对上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma, EIMS)的临床病理学特征的分析,提高对其诊断认识能力,减少误诊。方法：分析3例EIMS的临床病理特征并复习相关文献。结果：术后镜下示：肿瘤细胞弥漫分布,呈圆形、上皮样,胞浆丰富、嗜酸性、核偏位,核仁明显,部分可见梭形细胞,背景显著黏液变性伴突出的炎症细胞浸润,以中性粒细胞为主。免疫表型：肿瘤细胞表达ALK、Desmin、CD30、EMA、INI-1;部分表达：CK和SMA;不表达LCA、CD15、MyoD1、S-100、HMB-45、CD20、CD3、CD34、CD117和DOG-1。FISH检测显示3例均有ALK基因相关易位。结论：EIMS是高度恶性肿瘤,发生率比较低,细胞学形态特征、免疫组织化学特点及分子特征对本病的诊断具有重要价值。     

DOG-1在胃肠道间质瘤中的表达及其临床意义

目的 探讨DOG-1在胃肠道间质瘤(GIST)中的表达及其临床意义.方法 应用免疫组织化学EnVision法检测DOG-1在84例GIST患者肿瘤组织中的表达,并与CD117、CD34标记进行比较观察.结果 GIST患者手术切除肿瘤组织主要由数量不等的梭形细胞和上皮样细胞组成,两种细胞可按不同比例混合性或单一性组成肿瘤的实体.DOG-1、CD117和CD34在极低度及低度危险性GIST组织中阳性表达率分别为91.3%(42/46)、95.7%(44/46)和82.6%(38/46),在中度及高度危险性GIST组织中其阳性表达率分别为100%(38/38)、100%(38/38)和78.9%(30/38),对照组真性平滑肌瘤、神经鞘瘤、纤维瘤病及正常胃肠道黏膜组织中DOG-1、CD117、CD34均无表达,DOG-1标记GIST的敏感性及特异性与CD117相似,差异无统计学意义(P＞0.05),而标记中度及高度危险性GIST的敏感性与特异性明显高于CD34,其表达差异有统计学意义(P＜0.01).结论 DOG-1是一种新的诊断GIST比较特异的标记,尤其是对中度及高度危险性GIST比CD34有更高的敏感性和特异性.与CD117联合应用将有利于进一步提高GIST的诊断水平.

Abstract：

Objective To investigate the expression of DOG-1 in gastrointestinal stromal tumors (GIST)and its diagnostic application.Methods Immunohistochemical EnVision technique was used to assess the expression of DOG-1 in 84 cases of GIST in comparison with CD117 and CD34.Results All 84cases of GIST consisted of variable proportions of spindle and epithelioid tumor cells or iust one type of the tumor cell. The expression rates of DOG-1,CD117 and CD34 were 91.3%(42/46),95.7%(44/46)and 82.6%(38/46),in the group of very low and low risk GIST,and were 100%(38/38),100%(38/38)and 78.9%(30/38),respectively,in the group of moderate and hiish risk GIST. leiomyomas,schwannomas,fibromatosis and normal gastrointestinal mucoca did not express these markers.Moreover, the sensitivity and specificity of DOG-1 in the detection of GIST were similar to those of CD117.without statistical difference(P＞0.05)between the two markers.However,the sensitivity and specificity of DOG-1 detection of moderate and high risk GIsT were significandy higher than those of CD34(P＜0.01).Conclusions DOG-1 is a novel marker of gastrointestinal stromal tumors.It has the sensitivity and specificity hisher than CD34,especially in the detection of moderate and high risk GIST.Combined DOG-1and CD117 immunohistochemistry will likely improve the diagnostic accuracy of GIST.     

胃肠道间质瘤的光镜、免疫组织化学和超微结构的观察

目的 研究胃肠道间质瘤（GISTs）的光镜,电镜形态特点和免疫组织化学在诊断中的价值,探讨肿瘤的组织来源和分型。方法 对GISTs进行光镜和超微结构的观察,用EnVision二步法免疫组织化学方法检测波形蛋白,CD117（c-kit),CD34等8种抗原标记物在肿瘤中的表达情况。结果 65例GISTs占同期消化系统间叶性肿瘤的85．5％（65／76）;其中梭形细胞为主的有46例,伴有上皮样细胞的有13例,单纯由上皮样细胞组成的有6例,瘤细胞呈长,短梭形和圆形,胞质弱嗜酸,常见核端空泡,有时呈印戒样或透明细胞样;排列呈旋涡状,栅栏状或弥漫性巢状。超微结构表现出树枝样突起,神经内分泌颗粒,桥粒样连接等神经分化特点,或（和）胞质内出现密斑,密体等肌性分化。免疫组织化学显示肿瘤组织中抗原标记物表达阳性率波表蛋白为100％（65／65）,CD11793．8％（61／65）,CD3478．5％（51／65）。结论 GISTs是消化道最常见的间叶性肿瘤,光镜形态与真性肌源性和神经源性肿瘤极为相似,电镜和CD117,CD34等免疫标记物配合使用可对其做作出正确诊断,GISTs可能起源于多潜能的,卡哈尔间质细胞样的前体细胞。