胃肠道间质瘤中Ki-67表达及其与临床病理特征、危险度分级的相关性

目的探讨Ki-67在胃肠道间质瘤(gastrointestinal stromal tumors,GIST)中的表达及其与临床病理特征、危险度分级的相关性。方法分析77例手术切除的原发性GIST患者的临床病理资料,采用免疫组化SP法检测Ki-67的表达,分析其表达与GIST的临床病理特征及危险度分级的相关性。结果Ki-67表达在肿瘤部位、核分裂象、危险度分级中,差异有统计学意义(P0.05);在患者年龄、性别、肿瘤大小、组织类型、囊性变、单发或多发、出血、坏死及合并其他肿瘤中,差异无统计学意义(P0.05);Ki-67增殖指数与肿瘤部位、核分裂象、单发或多发及危险度分级有相关性(P0.05),其中Ki-67增殖指数与核分裂象及危险度分级呈正相关。结论Ki-67增殖指数可作为判断GIST危险度分级及预后预测的重要参考指标。     

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。     

CD117阴性的胃肠道间质瘤中DOG1的表达及意义

目的 探讨CD117阴性的胃肠道间质瘤(gastrointestinal stromal tumor,GIST)中DOG1的表达及意义.方法 通过免疫组化法检测58例CD117阴性的GIST中DOG1的表达.结果 DOG1的阳性表达率为93.1%(54/58).DOG1的表达与临床病理相关因素(性别、年龄、部位、肿瘤大小)无相关性(P>0.05).按危险程度分级分类:极低度8例,低度12例,中度21例,高度17例.DOG1的阳性率在不同危险程度间差异无显著性(P>0.05).结论 DOG1是比CD117更敏感、更特异的诊断GIST的免疫学指标,但不能作为判断其危险程度的指标.     

CD117、PDGFRA蛋白单独及联合DOG1检测在胃肠道间质瘤中的诊断价值

目的:探讨组织中CD117、PDGFRA两种蛋白表达在胃肠道间质瘤(gastrointestinal stromal tumors,GIST)中的诊断价值及联合DOG1蛋白检测的意义。方法:回顾性对99例GIST和25例非GIST肿瘤标本进行CD117、PDGFRA和DOG1蛋白表达进行检测并进行相关性分析。结果:GIST组CD117、PDGFRA和DOG1蛋白表达率分别为93.94%(93/99)、53.54%(53/99)和90.91%(90/99),非GIST组分别为4.00%(1/25)、4.00%(1/25)和12.00%(3/25),组间比较均有统计学意义(P<0.05);GIST组性别、年龄、肿瘤直径、肿瘤部位、组织学类型和危险度分级等临床病理参数与CD117、PDGFRA和DOG1蛋白表达无统计学意义(P>0.05);CD117、PDGFRA、DOG1、CD117和DOG1联合、PDGFRA和DOG1联合及三者联合判断GIST的敏感性分别为0.989、0.981、0.968、0.960、0.933和0.961,特异性分别为0.800、0.343、0.710、0.840、0.947和0.955,ROC曲线下面积(AUC)分别为0.945、0.748、0.895、0.895、0.840和0.975。结论:GIST中CD117、PDGFRA及DOG1蛋白表达在胃肠道间质瘤中的优势人群有待进一步研究;CD117、PDGFRA蛋白单独及联合DOG1检测可提高对GIST诊断的准确度。     

胃肠道间质瘤中LMTK3、caspase 3及caspase 7的表达意义

目的:探讨胃肠道间质瘤(GIST)中狐猴酪氨酸激酶3(LMTK3)、半胱氨酸天冬氨酸蛋白酶3(caspase 3)及半胱氨酸天冬氨酸蛋白酶7(caspase 7)蛋白的表达与该肿瘤临床病理特征的关系。方法:运用免疫组化EnVision二步法检测102例GIST患者肿瘤组织和相应正常组织中LMTK3、caspase 3及caspase 7蛋白的表达情况,分析LMTK3与caspase 3、caspase 7蛋白表达之间的关系,从而探讨3种蛋白表达与GIST临床病理特征之间的关系。结果:GIST肿瘤组织中LMTK3蛋白表达高于相应正常组织,差异具有统计学意义(P<0.05);LMTK3蛋白表达在不同肿瘤大小、核分裂计数及危险度分组之间比较差异有统计学意义(P<0.05),在不同患者性别、年龄及肿瘤发生部位分组之间差异无统计学意义(P>0.05);肿瘤组织中caspase 3和caspase 7蛋白表达均低于相应正常组织,差异具有统计学意义(P<0.05);caspase 3与caspase 7蛋白表达在患者不同性别、年龄、肿瘤发生部位、肿瘤大小、核分裂像计数及危险度...     

胃肠间质瘤预后因素分析

目的：探讨胃肠间质瘤(GIST)的临床特点、诊断治疗及影响预后的可能因素,为判断患者病情和制定诊疗方案提供依据。方法回顾分析2007年7月~2013年7月兴化市人民医院收治经手术治疗并经病理确诊的63例GIST的临床资料,记录性别年龄、临床表现、相关检查、肿瘤性质、CD117、CD34、S-100、NSE、Ki-67指数等信息,分析其与GIST危险度分级的相关性。结果临床多见的症状为腹痛,肿瘤最多见发生部位为胃,其次为小肠。免疫组化检测CD117、CD34、S-100、NSE、Ki-67的阳性表达率分别为100%,74.2%,11.9%,32.6%,57.1%。影响总体生存时间的因素是肿瘤大于5cm、有丝分裂数>5/50HPF、肿瘤转移、手术切除不完全、Ki-67过度表达。结论①GIST患者临床症状无特异性,诊断依靠常规病理与免疫组化的联合检测,CD117是特异性指标,在大多数胃肠间质瘤中表达;②GIST主要治疗方法为手术,肿瘤应尽可能完整切除,避免肿瘤破裂,Imatinib可明显改善GIST预后;③影响GIST预后主要因素有：肿瘤大小、有丝分裂、转移、能否完全切除、Ki-67的表达。     

胃肠道间质瘤33例临床病理分析

目的 分析胃肠道间质瘤(gastrointestinal stromal tumors,GISTs)的免疫组化表型特点,提高GISTs的诊断准确性;探讨GISTs的危险程度分级与肿瘤大小、核分裂象及Ki-67增殖指数的关系.方法 回顾性分析33例GISTs的临床特征及病理组织形态、免疫表型.结果本组33例中危险程度极低度2例、低度8例、中度4例、高度16例,3例未能分级.免疫表型:CD117阳性27例、CD34阳性27例、NES阳性25例、vimentin阳性33例、S-100阳性5例、desmin阳性4例、HHF35阳性11例、SMA阳性11例、CK(AE1/AE3)阳性0例.结论 GISTs常发生于胃、小肠,其中小肠的恶性度最高.联合应用CD117、CD34、NES、S-100等免疫组化标记物可以提高GISTs的诊断准确性.GISTs的恶性程度与肿瘤大小、核分裂象有密切的关系.细胞增殖活性Ki-67增殖指数增高,肿瘤的恶性程度亦增高.     

胃肠道间质瘤124例DOG1、WISP-1表达与临床意义

目的 探讨DOG1和WISP-1在胃肠道间质瘤(gastrointestinal stromal tumors,GIST)中诊断及预后的意义.方法 收集川北医学院附属医院病理科124例有完整临床病理资料的GIST,应用免疫组化EnVision两步法检测了DOG1和WISP-1蛋白在124例GIST中的表达情况,并与非GIST进行对照研究;应用χ2检验及Spearmen检验对结果进行统计学分析.结果 124例GIST中DOG1的阳性表达率为96%(119/124),CD117的阳性变表达率为91.0%(111/124),两者在GIST中的表达水平差异无统计学意义(P>0.05);GIST与非GIST中DOG1的表达水平差异有统计学意义(P<0.001);本组病例中WISP-1阳性率为80.6%(100/124),其中极低风险性、低风险性、中风险性和高风险性的阳性表达率分别为44.4%(4/9)、66.7%(26/39)、86.9%(17/20)和95.0%(38/40),其阳性表达率与美国国立卫生研究院(NIH)风险分级呈正相关关系(P<0.001);GIST与非GIST 中WISP-1的表达水平差异无统计学意义(P>0.05).结论 DOG1是GIST中一个敏感又特异的标记物,与CD117联用能提高GIST的诊断准确率,临床上可将其作为鉴别消化道间叶源性肿瘤的一线抗体;WISP-1的表达可能和GIST恶性进程有关,有可能作为评估GIST生物学行为的指标.     

胃肠道间质瘤中DOG1和nestin的表达及意义

目的:探讨DOG1和nestin在胃肠道间质瘤(gastrointestinal stromal tumor, GIST)中的表达及其诊断价值.方法:采用免疫组织化学EnVision法分别检测25例GIST、5例平滑肌瘤和5例神经鞘瘤中DOG1和nestin的表达.结果:DOG1和nestin在GIST中的阳性表达率分别为100%和88%,而CD117和CD34在GIST中的阳性表达率分别为84%和64%.GIST中DOG1和nestin的表达与肿瘤部位、肿瘤大小、危险度分级和组织学形态均无关(P>0.05).5例平滑肌瘤中DOG1和nestin均阴性表达,DOG1和nestin在神经鞘瘤中的阳性表达率分别为0和100%.DOG1,nestin,CD117和CD34联合检测阳性率为48%.结论:DOG1在诊断GIST中具有敏感性和特异性,而nestin则是GIST诊断中比较敏感的一种标志物,但特异性差.DOG1,nestin,CD117和CD34联合检测可提高GIST的正确诊断.     

胃肠道间质瘤64例免疫标记物应用分析

目的 探讨胃肠道间质瘤(gastrointestinal stromal tumor,GIST)的免疫组化标记物的表达特征,为其诊断及鉴别诊断提供依据.方法 收集85例确诊的胃肠道及腹、盆腔间叶源性肿瘤标本,其中GIST 64例.免疫组化法检测CD117、CD34、DOG1、NSE、S-100、SMA和vimentin的表达.结果 GIST组中CD117、CD34、DOG1、NSE阳性率分别为89.1%、76.6%、96.9%及87.5%;非GIST组中的阳性率分别为4.8%、33.3%、28.6%和50.0%.CD117阴性的GIST组中CD34、NSE、DOG1阳性率分别为28.6%、71.4%和85.7%.结论 GIST中DOG1表达的敏感性高于CD117,在CD117阴性的病例中DOG1优于其他标记物.DOG1与CD117及NSE等联合应用于免疫组化检测足以明确绝大多数GIST的诊断.     

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。     

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.     

Synchronous GIST with osteoclast-like giant cells and a well-differentiated neuroendocrine tumor in Ampula Vateri: coexistence of two extremely rare entities

Mesenchymal tumors of the gastrointestinal system with variable histopathological appearances and constant expression of CD117 are known as gastrointestinal stromal tumors (GISTs). Neuroendocrine tumors may be seen in the gastrointestinal system and other organ systems of the body. We report a 44-year-old male patient with a 6.5 x 3 x 6cm mass located in the Ampulla of Vater. Histopathologic examination revealed a GIST with a marked nuclear pleomorphism and a high mitotic rate, and that was rich in osteoclast-like giant cells (OGC). Immunohistochemically, GIST was positive for CD117, while OGCs were negative for CD117 and positive for CD68 and alpha1-antitrypsin. There was also found a well-differentiated neuroendocrine tumor near the GIST, in the serosal aspect of the duodenum at the point of the Ampulla of Vater. This second tumor was 20mm in diameter, and was relatively well circumscribed with few glands invading the GIST. This tumor was positive for synaptophysin and chromogranin. Neither mitosis nor vascular invasion was observed. The patient had no familial history or clinical manifestations of neurofibromatosis. This case presents the unique synchronous existence of two extremely rare entities, a GIST with OGC and a well-differentiated neuroendocrine tumor, both located in the Ampulla of Vater.     

The intermediate filament protein synemin (SYNM) was found to be more widespread in CD117+ gastrointestinal stromal cell tumors (GIST) than the CD34 transmembrane phosphoglycoprotein: an immunohistochemical study

Interstitial cells of Cajal (ICC) are the pacemaker cells in the gastrointestinal system, initiating the rhythmic systematic contraction of smooth muscle to regulate peristalsis. Benign ICC are identified immunohistochemically by positive staining for CD117, c-kit tyrosine kinase receptor. CD117 expression is maintained during the malignant transformation of ICC into gastrointestinal stromal cell tumors (GIST). However, to date, no single reliable marker for GIST has been identified by immunohistochemical (IHC) staining, and correct diagnosis depends on IHC staining results using multiple markers. This study compared the reactivities of synemin (SYNM) intermediate filament protein and CD34, a known marker for stem cells, endothelial cells, and many GIST tumors, in 54 formalin-fixed, paraffin-embedded GIST, which were determined by this study to be CD117 positive. Double-immunofluorescence (IF) staining was also used to assess whether CD117 and SYNM were co-expressed by ICC. While 81.5% of the GIST were CD34⁺, 92.9% of these tumors were SYNM⁺. ICC were CD117⁺/SYNM⁺, whereas mast cells were CD117⁺/SYNM^?. Because the percentage of CD117⁺/SYNM⁺ GIST was higher than the percentage of 117⁺/CD34⁺ GIST, this study suggested that SYNM was a better marker than CD34 for GIST diagnosis. In addition, differential expression of SYNM and CD117 helped distinguish between ICC and mast cells.     

Immunohistochemical comparison of gastrointestinal stromal tumor and solitary fibrous tumor

CONTEXT: The differential diagnosis of gastrointestinal stromal tumors (GIST) and solitary fibrous tumors (SFT) may be a diagnostic challenging because of overlapping clinicopathologic features. Many studies have shown consistent immunoreactivity for CD117 (c-Kit) in GIST. However, only a few studies have evaluated CD117 expression in SFT, and these studies have used an antibody from Santa Cruz Biotechnology. In non-GIST lesions, reactivity with this antibody has been shown to differ from that with a CD117 antibody from Dako Corporation. The immunoreactivity of SFT with the Dako CD117 antibody has not been reported. Conversely, CD99 is a marker for SFT, and its expression in GIST has not been evaluated. OBJECTIVE: To study the immunohistochemical profiles of GIST and SFT to evaluate their diagnostic overlap. DESIGN: We studied the immunoreactivity of 27 unequivocal GIST and 19 unequivocal extra-abdominal SFT for CD117, CD34, CD99, alpha-smooth muscle actin, vimentin, CD31, S100 protein, and muscle-specific actin. All antibodies, including CD117, were from Dako Corporation. RESULTS: We found positive immunoreactivity for CD117 in 100% of GIST and none of SFT; for CD34 in 89% of GIST, and 100% of SFT; for CD99 in 89% of GIST and 100% of SFT; for alpha-smooth muscle actin in 48% of GIST and 31% of SFT; for vimentin in 89% of GIST and 90% of SFT; and for muscle-specific actin in 22% of GIST and none of SFT. None of the GIST or SFT showed immunoreactivity for CD31 and S100 protein. CONCLUSIONS: The major difference between GIST and SFT was strong CD117 immunoexpression in all GIST and an absence of this expression in all SFT. With the exception of muscle-specific actin, the prevalence of immunoreactivity for the markers studied did not differ substantially between these 2 tumors. We conclude that GIST and SFT show distinctly divergent immunoprofiles with respect to CD117 and muscle-specific actin.     

77例胃肠道间质肿瘤的病理形态学及免疫组化研究

目的：研究胃肠道间质瘤（GIST）的病理形态及免疫组化特点,方法：应用光镜观察77例GIST的形态特征,用免疫组化S－P法检测c-kit(CD117),CD34,vimentin,SMA及S－100蛋白在GIST中的表达情况。结果：GIST的瘤细胞较经典的平滑肌瘤更丰富,胞质嗜酸较弱,瘤细胞为酸形或上皮样,或酸形与上皮样细胞混合存在,胞质内常见空泡形成;排列成交织刺状、弥散片状、栅栏状或轮辐状、较为特征的是细胞团巢形成。常见间质或见管壁玻变。原发于肠系膜者恶性潜力较高。CD117和CD34的阳性率分别为90％和92％,结论：胃肠道间质肿瘤有较为特独的组织学形态,CD117和CD34联合使用可协助鉴别诊断。     

胃肠道间质瘤病理及CD117和CD34表达与意义

目的：对本院病案资料中GIST重新分类，加 强对胃肠道间质瘤的认识。方法：用CD117、CD34、α-SMA、S-100等抗 体对56例GIST进行标记、分类，根据GIST的临床病理及形态学特点，正确诊断，并探讨 GIST的临床特点。结果:56例GIST CD117、CD34弥漫强表达，抗体 阳性率分别为CD117（50/56，89.3%）、CD34（37/56，66.1%），部分病例局灶表达α-SM A、S-100，阳性率分别为（17/56，30.4%）、（4/56，7.1%），结蛋白desmin均阴性。其 中良性及交界性29例，恶性27例。本组病例中胃及小肠GIST共达91.1％，其它部位少见。 结论：CD117、CD34在GIST表达显著，可作为GIST诊断的一个辅助指标。 胃和小肠GIST最常见，胃镜等影像学无特异性，诊断主要依靠病理诊断。组织病理不仅有助 于诊断，而且有助于预后的判断。     

Gastrointestinal Kaposi's sarcoma: CD117 expression and the potential for misdiagnosis as gastrointestinal stromal tumour

Aims:  Gastrointestinal Kaposi's sarcoma (KS) may mimic gastrointestinal stromal tumours (GISTs) histologically. Studies have shown that KS outside the gastrointestinal (GI) tract may express CD117, an antibody usually used to support a diagnosis of GIST. The aim was to evaluate the clinicopathological features of GI KS, including the expression of CD117 with and without antigen retrieval.
Methods and results:  Fourteen GI KS were assessed histologically, 12 of which were also subjected to immunohistochemistry for CD34, human herpesvirus (HHV) 8, DOG1 and CD117. CD117 immunohistochemistry was performed with and without antigen retrieval. All cases showed an infiltrative spindle cell tumour. Lamina propria infiltration, lymphoplasmacytic inflammation, extravasated red blood cells and haemosiderin were typical histological features. In all cases tumour cells were positive for CD34 and HHV8, but negative for DOG1. CD117 was positive in four of 12 cases without antigen retrieval and 10 of 12 cases with antigen retrieval.
Conclusions:  The microscopic distinction of GI KS from GIST can be difficult. Clues that raise the possibility of GI KS include young patient age, a history of immunosuppression, lamina propria infiltration, lymphoplasmacytic inflammation, extravasated red blood cells and haemosiderin deposition. Use of the immunomarkers CD117 (without antigen retrieval), HHV8 and DOG1 may aid in the distinction between GI KS and GIST.     

29例上皮样胃肠间质瘤的临床病理特征

目的:探讨29例上皮样胃肠间质瘤(gastrointestinal stromal tumors,GIST)的组织学形态和免疫组织化学特点以及诊断和鉴别诊断,对临床正确诊断治疗和判断预后具有十分重要的临床意义.方法:回顾性分析安阳市肿瘤医院病理科2009年月3月至2016年8月321例完整切除GIST标本,经筛查并重新阅片诊断上皮样GIST 29例.结果:29例上皮样GIST,发生胃部15例,小肠2例,肠系膜3例,直肠4例,腹腔3例,腹膜后1例,盆腔1例.恶性GIST 25例,良性4例.瘤细胞丰富,胞质嗜酸或透明,部分瘤细胞核质比高,核大小不等具有多形性,核分裂较多,可伴有多灶凝固性坏死,间质多数伴有黏液样变性.组织结构形成器官样、片状、巢状及腺泡状等.免疫组织化学CD34,DOG-1在上皮样GIST均弥漫阳性(阳性率100%),CD117阳性率(86%).结论:上皮样GIST发生部位广泛,形态多变,易误诊其他上皮样分化的肿瘤;免疫组织化学CD34,DOG-1,CD117在上皮样GIST诊断及鉴别诊断中具有重要价值;如肿物较大、细胞丰富、核分裂多、间质黏液样变性;绝大多数要考虑恶性GIST.     

Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report

Carney triad is a rare syndrome, with only 20 complete cases reported. We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma. Immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma. Ultrastructural studies demonstrated skeinoid fibers in the GIST. To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.