66例胃肠道间叶源性肿瘤临床和病理特征分析

背景：胃肠道间质瘤(GISTs)是发生于胃肠道的一种特殊类型的间叶源性肿瘤，具有从良性到显著恶性的广谱生物学行为．其特征性分子学改变是c—kit基因突变，其他胃肠道间叶源性肿瘤(GIMTs)则无此特征。因此，有必要正确认识和诊断GISTs．以将其与其他GIMTs区分开来。目的：加强对GISTs的认识，以正确诊断GISTs。方法：总结1980—2004年间杨浦区中心医院GIMTs病例的病史资料，选用CD117、CD34、α-平滑肌抗体(SMA)、结蛋白(desmin)和S-100这一组抗体对GIMTs进行标记、分类，重点分析GISTs的临床、病理和免疫组化特征。结果：本组66例GIMTs中56例为GISTs(84．8％)，肿瘤多发生于胃和小肠(91．1％)，良性和交界性29例，恶性27例，临床表现主要为中上腹不适、腹部包块和消化道出血。间质瘤组织由梭形细胞和上皮样细胞组成，CD117和CD34呈弥漫性强表达，阳性率分别为89．3％和66．1％．部分病例局灶表达α-SMA和S-100，阳性率分别为30．4％和7．1％，desmin表达均阴性。平滑肌瘤3例，均弥漫性强表达α-SMA和desmin，余阴性。神经鞘瘤4例，均弥漫性表达S-100，余阴性。其他少见或难以分类的肿瘤3例。结论：本组GIMTs中以GISTs最为多见，平滑肌瘤和神经鞘瘤少见。临床丁作中应加强对GISTs临床和病理特征的认识，以提高其诊断和治疗水平。     

265例胃肠道间叶源性肿瘤的临床病理特征及超声内镜诊断价值

目的探讨胃肠道间叶源性肿瘤（gastrointestinal mesenchymal tumor,GIMT）的临床病理特征及超声内镜（edoscopic ultrasonography,EUS）的诊断价值.方法观察265例GIMT病理特征并检测CD117、CD34、平滑肌肌动蛋白（SMA）、S-100、Ki-67等抗体的表达情况,确诊后回顾其中32例术前EUS检查结果.结果 265例GIMT中胃肠道间质瘤（gastrointestinal stromal tumor,GIST）146例,平滑肌（肉）瘤（leiomyoma or leiomyosarcoma）113例,神经源性肿瘤6例.免疫组化结果：GIST以CD117阳性132/146（90.4%）和CD34阳性109/146（74.7%）为主,SMA和S-100分别在平滑肌（肉）瘤和神经鞘膜瘤中强阳性表达,9例GIST中7例Ki-67阳性且伴较多有丝分裂,病理诊断为交界性或恶性GIST.交界性、恶性GIST多见于男性患者.EUS对GIST、平滑肌瘤的定位准确率为96.9%,诊断准确率84.4%,良恶性鉴别准确率71.9%.结论 GIMT主要为GIST.形态上类似的GIST与平滑肌瘤及神经鞘膜瘤区别可用CD117、CD34、SMA、S-100等多种免疫组化标记物.联用Ki-67表达和有丝分裂数判断间质瘤的良恶性的敏感性、特异性高.EUS对于GIMT的诊断及良恶性鉴别有一定的应用价值,结合EUS引导下细针穿刺（EUS-FNA）活检是未来的诊断选择.     

小探头超声在上消化道间叶源性肿瘤的诊断及指导治疗中的价值分析

胃肠道间叶源性肿瘤（gastrointestinal mesenchymal tumor,GIMT）是指从食管到直肠的消化道非上皮淋巴造血细胞呈梭形细胞分化为主的软组织肿瘤,包括胃肠道间质瘤、平滑肌肿瘤、神经鞘瘤、脂肪源性肿瘤、胃肠道自主神经肿瘤等。     

53例胃肠道间叶性肿瘤内镜特点及病理研究

目的 观察53例胃肠道间叶性肿瘤的内镜和病理特点,加强对间叶性肿瘤的鉴别诊断.方法 收集53例胃肠道间叶性肿瘤患者的影像、病理资料,并完成CD117、CD34、SMA和Desmin的免疫组化检查,分析胃肠道间叶性肿瘤的临床、内镜、病理和免疫组化特征.结果 间质瘤和平滑肌源性肿瘤在内镜超声中有一定的影像学特点;胃和食管发生间质瘤和平滑肌瘤比例高于消化道其他部位,分别为64.71%和68.42%;间质瘤中CD117和CD34为弥漫性阳性表达,SMA和Desmin阳性表达率较低,但在小肠病例中SMA表达较CD34高;平滑肌源性肿瘤中SMA和Desmin呈弥漫阳性表达,而CD117和CD34的表达显著低于间质瘤;同时根据相关性分析证实,Desmin阴性对间质瘤的诊断意义增强,CD117和CD34阴性有利于平滑肌瘤的诊断.结论 内镜超声检查结合CD117、CD34、SMA和Desmin的联合检测可以提高对梭状细胞形态的间叶性肿瘤的诊断准确性.     

胃肠道间质瘤102例临床预后因素分析

胃肠道间质瘤(GIST)以往被误诊为平滑肌瘤或平滑肌肉瘤, 近年基于免疫组化及电镜的发展被区分出来, 是较常见的间叶源性肿瘤,可发生在消化道的各个部位,有关其预后的文献报道相对较少.现收集河北医科大学第四医院1996年7月至2007年6月接受手术治疗并经病理证实的胃肠道间质瘤102例,探讨影响胃肠道间质瘤预后的因素.     

光镜诊断383例平滑肌肿瘤组织发生学的重新评价

目的重新评价胃肠道平滑肌肿瘤的组织发生。方法对38例光镜诊断胃肠道平滑肌肿瘤用组织化学和免疫组化方法。组合分类判别各例组织的来源。结果发现光镜诊断胃肠道平滑肌肿瘤并非单一平滑肌源性(39.4%),还有神经源性(13.2%),混合性(42.1%)及未分化性(5.3%)。结论作者认为应将胃肠道平滑肌肿瘤归为胃肠道间质瘤的范畴,再用免疫组化标记及电镜阐明组织发生,为临床治疗和判断预后提供更准确的依据。     

超声内镜辅助胃食管间叶源性肿瘤的内镜治疗25例

目的 探讨在超声内镜(endoscopicultrasonography,EUS)辅助下,针对胃及食管间叶源性肿瘤的个性化治疗方法.方法 患者选择标准:(1)最大直径＜ 2.0 cm起源于黏膜肌层的黏膜下肿瘤(submucosal tumor,SMT).(2)最大直径＜1.2 cm起源于固有肌层的SMT.(3)肿瘤向腔内生长.(4)无肿瘤转移的其他影像学证据.入选病例25例,其中男15例,女10例.采用内镜下黏膜切除术(endoscopic mucosal resection,EMR)方法治疗黏膜肌层的肿瘤,固有肌层的肿瘤采用结扎方法.结果 6例病变位于胃固有肌层的SMT(胃底4例,胃体1例,胃窦1例)采取内镜下结扎方法治疗;19例食管黏膜肌层SMT采取内镜下EMR治疗.25例治疗过程中均未发生出血及穿孔,术后复查超声内镜创面愈合,病变无残留.结论 EUS在选择间叶源性肿瘤治疗方式方面具有积极的作用,可为病人选择个性化治疗,使内镜下治疗更安全、有效.起源于固有肌层的肿瘤,采用内镜下结扎治疗,与内镜下剥离治疗相比,创伤小、并发症少,不失为一种好的治疗办法.     

微探头超声对上消化道间叶源性肿瘤的诊断价值探讨

目的 探讨微探头超声对上消化道间叶源性肿瘤(GIMT)的诊断价值.方法 回顾分析38例进行微探头超声检查的GIMT患者的EUS特征,并将检查结果与术后病理结果进行对照.结果 38例GIMT患者EUS诊断间质瘤25例,平滑肌瘤11例,平滑肌肉瘤2例.病理组织学及免疫组织化学检查后确诊间质瘤28例,其中中、高度恶性间质瘤6例;平滑肌瘤8例,平滑肌肉瘤1例,神经纤维瘤1例.EUS诊断准确率达89%.结论 EUS能较好鉴别GIMT与其它黏膜下肿瘤,是目前诊断黏膜下肿瘤较为准确有方法.     

下消化道间叶源性肿瘤CT仿真内镜与病理的对照研究

目的 观察下消化道问叶源性肿瘤(GIMTs)的病理及免疫组化特点,对照研究其与CT仿真内镜(CTVE)诊断之间的关系,评价CTVE在下消化道GIMTs中的诊断价值.方法 收集74例下消化道GIMTs患者的手术病理标本,采用光镜观察其病理特点及良恶性状况,免疫组化法检测其CD117、CD34、α-平滑肌抗体(SMA)及S-100蛋白的表达,并与术前CTVE判定的病变部位及良恶性结果进行对照研究.结果 经病理及免疫组化检查,40例(54.1%)诊断为胃肠道间质瘤(GIST),其中恶性间质瘤16例(40%);33例(44.6%)诊断为平滑肌瘤;1例(1.4%)诊断为神经鞘瘤.发病部位位于空肠33例,回肠21例,大肠20例.免疫组化:CD117阳性38例,占51.4%;CD34阳性27例,占36.5%;SMA阳性46例,占62.2%,S-100阳性1例(1.4%).CTVE对病变部位准确定位69例(93.2%).其中大肠准确定位18例,符合率90.0%;空回肠准确定位51例,符合率94.4%.CTVE判断良恶性GIST的敏感性为84.2%,特异性为85.7%.结论 GIST是下消化道最常见的GIMTs,发病部位以小肠居多.CTVE能准确显示肿瘤的部位、形态、大小,可术前准确定位GIMTs,对其良恶性判断具有较高的敏感性和特异性,可为术前制定合理手术方案和治疗策略提供重要依据.     

下消化道间叶源性肿瘤CT仿真内镜与病理的对照研究

目的 观察下消化道问叶源性肿瘤(GIMTs)的病理及免疫组化特点,对照研究其与CT仿真内镜(CTVE)诊断之间的关系,评价CTVE在下消化道GIMTs中的诊断价值.方法 收集74例下消化道GIMTs患者的手术病理标本,采用光镜观察其病理特点及良恶性状况,免疫组化法检测其CD117、CD34、α-平滑肌抗体(SMA)及S-100蛋白的表达,并与术前CTVE判定的病变部位及良恶性结果进行对照研究.结果 经病理及免疫组化检查,40例(54.1%)诊断为胃肠道间质瘤(GIST),其中恶性间质瘤16例(40%);33例(44.6%)诊断为平滑肌瘤;1例(1.4%)诊断为神经鞘瘤.发病部位位于空肠33例,回肠21例,大肠20例.免疫组化:CD117阳性38例,占51.4%;CD34阳性27例,占36.5%;SMA阳性46例,占62.2%,S-100阳性1例(1.4%).CTVE对病变部位准确定位69例(93.2%).其中大肠准确定位18例,符合率90.0%;空回肠准确定位51例,符合率94.4%.CTVE判断良恶性GIST的敏感性为84.2%,特异性为85.7%.结论 GIST是下消化道最常见的GIMTs,发病部位以小肠居多.CTVE能准确显示肿瘤的部位、形态、大小,可术前准确定位GIMTs,对其良恶性判断具有较高的敏感性和特异性,可为术前制定合理手术方案和治疗策略提供重要依据.

Abstract：

Objective To study the pathological and immunohistochemical features of alimentary tract mesenchymal tumors and compare with computed tomographic virtue endoscopy (CTVE) imaging technology to evaluate the diagnostic value of CTVE in alimentary tract mesenchymal tumors. Methods Seventy-four pathological specimens of alimentary tract mesenchymal tumors were collected. The pathological features and the expression of CD117, CD34, SMA and S-100 were observed by immunohistochemical method with light microscope. The pathological types and characteristics were determined by pathologists and compared with CTVE imaging technology. Results In the 74 cases of alimentary tract mesenchymal tumors,40 cases were diagnosed as stromal tumor with pathological and immunohistochemical methods (54. 1%).Sixteen of them were malignant, accounting for 40% of the stromal tumor while 33 cases were diagnosed as leiomyoma(44. 6%)and 1 case as schwannoma(1.4%) . In the 74 GIMTs cases ,33 were jejunum GIMTs,21 were ileum GIMTs and 20 were large intestine GIMTs. Immunohistochemistry assay in the 74 GIMTs cases showed that 51.4% GIMTs were positive for CD117, approximately 36. 5% were positive for CD34 , 62.2% were positive for smooth-muscle actin (SMA) and 1. 4% were positive for S-100 protein. In the 74 GIMTs cases,69 cases were diagnosed right in the accuracy for location with CTVE(93. 2%) with 51 cases in small intestinal (94. 4%) and 18 cases in large intestinal (90. 0%). The sensitivity and the specificity of CTVE to distinguish benign from malignant stromal tumors by CTVE characteristics were 84. 2% and 85. 7%respectively. Conclusions GIST is common in GIMTs and is often originated from the small intestinal. The immunohistochemistry has great value in diagnosing alimentary tract mesenchymal tumors. The CTVE imaging technology also has great value in diagnosing alimentary tract mesenchymal tumors which can show the localization, shape size and artery of the tumor clearly. The diagnostic sensitivity and specificity of CTVE are high to distinguish benign from malignant alimentary tract GISTs. CTVE plays an important role in guiding the clinical management of GISTs.     

胃肠间质瘤的临床及病理特征分析138例

目的:探讨胃肠道间质瘤(gastrointestinal stromal tumors,GIST)的临床、病理和免疫组织化学特征.方法:收集澳门仁伯爵医院及北京医院2002-01/2008-07资料完整GIST患者138例的临床、手术及病理资料、免疫组织化学测得的CD117、CD34、SMA、Desmin、S100蛋白表达结果,回顾性分析GIST临床与病理的特点及关系.结果:138例间质瘤位于胃90例(65.22%),小肠36例(26.09%),结直肠9例(6.52%),腹膜后3例(2.17%);最大直径1-14cm.瘤组织由梭形细胞和/或上皮样细胞构成.梭形细胞:形态较一致,呈长梭形,胞质丰富,嗜酸性,胞核呈杆状或腊肠样或长梭形,大多数似平滑肌细胞,少许似神经鞘或神经纤维细胞;上皮样细胞:体积较大,形态不一,呈多角形或卵圆形,胞质空亮或微嗜酸性,核周出现亮区,核形多样与核分裂数成正比.梭形细胞型114例(82.61%),上皮样细胞型6例(4.35%),混合型18例(13.04%);核分裂小于或等于5个/50HPF99例,5-10个/50HPF24例,大于10个/50HPF15例.免疫组织化学阳性表达率为CD11797.38%,CD3473.91%,SMA6.52%,Desmin2.17%,S1002.17%;良性肿瘤24例(17.39%),低度恶性潜力39例(28.26%),中度恶性潜力33例(23.91%),高度恶性潜力21例(15.22%),恶性21例(15.22%).结论:GIST具有独特的临床特征;CD117对GIST的诊断具有较高的特异性和敏感性,但与CD34、SMA、Desmin、S100等同时检测有助于GIST的诊断;GIST良恶性的评估取决于肿瘤的部位、大小和核分裂数的多少.     

胃肠道间质瘤的临床病理和免疫组织化学特征

目的探讨胃肠道间质瘤(GIST)的临床病理以及免疫组织化学特征。方法回顾性分析72例GIST患者的临床资料。结果 72例GIST患者的肿瘤部位为:25例(34.7%)胃部,24例(33.3%)小肠,23例(31.9%)其他部位。镜下显示主型为梭形细胞68例(94.4%),主型为上皮样细胞1例(1.4%),混合细胞型3例(4.2%)。免疫组化检查显示,71例(98.6%)CD117阳性,52例(72.2%)CD34阳性,22例(30.6%)SMA阳性,1例(1.4%)Desmin阳性,6例(8.3%)S-100阳性。结论 GIST的发病部位较多,组织形态学表现复杂,联合应用免疫组化检查有助于临床诊断与鉴别。     

肝转移性胃肠道间质瘤临床病理分析15例

目的:探讨肝转移性胃肠道间质瘤(GISTs)的临床病理、免疫组化特点.方法:复习文献,分析15例肝转移性GISTs的临床表现、组织学及免疫组化特征.结果:本组15例肝转移性GISTs,原发灶分别位于小肠(6例)、胃(5例)及网膜(2例),余2例来源不明.发现肝转移灶与原发肿瘤手术相距时间为0 d-86 mo(平均25 mo±9 mo).肿瘤大小为1.5-30.0(平均7.1±1.2)cm.6例伴有出血、坏死或囊性变.组织学分型:梭形细胞型11例,上皮样细胞型2例,混合型2例.免疫组化结果:肿瘤细胞弥漫表达CD117(86.7%)、CD34(46.7%),灶性表达SMA(33.3%)、S-100(20%),Desmin均为阴性.结论:肝脏是GISTS最常见的转移部位,原发部位多为小肠和胃.其临床表现、组织形态及免疫组化表型与原发灶基本一致.预后较差.CD117和CD34联合应用有助于鉴别诊断.     

Expressions of sonic hedgehog, patched, smoothened and Gli-1 in human intestinal stromal tumors and their correlation with prognosis

INTRODUCTION Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the gastrointestinal tract that occur from the esophagus to the anus, including in the omentum, mesentery and retroperitoneum[1]. Small GISTs are often detected during surgery for other conditions, gastroscopy or routine X-ray[1,2]. Some GISTs present with bleeding, perforation, pain, obstruction or a combination of these symptoms[3,4]. These tumors have a wide clinical spectrum from benign, incidenta…     

Esophageal mesenchymal tumors： Endoscopy, pathology and immunohistochemistry

AIM： To study the endoscopic, pathological and immunoo histochemical features of esophageal mesenchymal tumors. METHODS： Twenty-nine patients diagnosed as esophageal rnysenchymal tumors by electronic endoscopy and endoscopic ultrasound （EUS） were observed under light microscopes, and all tissues were stained by the immunohistochemical method. The expression of CD117, CD34, SMA and desmin were measured by staining intensity of cells and positive cell ratios. RESULTS： Endoscopically, esophageal gastrointestinal stromal tumors （GISTs） and leiomyomas （LMs） had similar appearances, showing submucosal protuberant lesions. They all showed low echo images originated from the muscularis propria or muscularis mucosa on EUS. Endoscopy and EUS could not exactly differentiate esophageal GISTs from LMs. Microscopically, there were two kinds of cells： spindle cell type and epitheloid cell type in esophageal GISTs. Leiomyomas and leiomyosarcornas were only of spindle cell type. One malignancy was found in five cases of esophageal GISTs, and one malignancy in 24 cases of leiomyomas and leiomyosarcomas. Using Fisher＇s exact method, the differences of malignant lesion proportion were not significant between esophageal LMs and GISTs, 1/5 vs 1/24 （P 〉 0.05）. All cases of esophageal GISTs were positive for CD117, and 3 cases were also positive for CD34. The 24 cases of leiomyomas and leiomyosarcomas were all negative for CD117 and CD34. The differences of positive rates of CD117 and CD34 were significant between esophageal GISTs and LMs, 5/5 vs 0/24, 3/5 vs 0/24 （P 〈 0.005）. All leiomyomas and leiomyosarcomas were positive for SMA, and desmin. Among 5 cases of esophageal GISTs, 2 cases were SMA positive, and 1 case was desmin positive. The differences in positive rates and expression intensity of SMA and desmin were significant between esophageal LMs and GISTs, 24/24 vs 2/5, 24/24 vs 115 （P 〈 0.005）. CONCLUSION： The most common esophageal mesenchymal tumors are leiomyomas, and esophageal GISTs     

26例胃肠道间质瘤肝转移的临床特征和生存分析

目的 总结胃肠道间质瘤肝转移的临床病理特征，行生存分析，对诊断和治疗原则进行探讨。方法 收集本院收治的资料完整的胃肠道间质瘤患者99例，对其中26例肝转移患者的临床病理特征进行回顺性分析，探讨肝转移发生后生存时间的影响因素。结果 原发肿瘤和发生肝转移的平均年龄为50．8和51．8岁。病理证实者5例，手术中探查诊断为肝转移者12例，14例为单纯影像学诊断。发现原发肿瘤的同时和非同时伴肝转移者分别为8例和18例，非同时发现者肝转移距原发肿瘤手术时间的中位时间为12个月。肿瘤原发于胃12例．结直肠5例，小肠6例，胃肠道外3例。肝转移灶切除者4例，无水乙醇注射2例，介入治疗3例，全身静脉化疗7例，伊马替尼（格列卫）治疗8例，未治疗2例。肝转移后中位生存时间为21个月。伊马替尼治疗为影响肝转移后生存时间的重要因素。结论 原发部位为胃的胃肠道间质瘤肝转移最常见；小肠问质瘤易发生肝转移；服用伊马替尼3个月以上可延长生存。     

Endoscopic enucleation of gastrointestinal stromal tumors of the stomach： Report of five cases

Gastrointestinal stromal tumor （GIST） of the stomach was treated by endoscopic enucleation in five patients. They were three men and two woman, aged 36-56 years. Tumors located in the cardia were completely enucleated endoscopically without any serious complication. The largest diameter of removed tumors ranged from 1.2 to 2.5 cm. Histopathological diagnosis was GIST with low risk of malignancy （mitotic index 〈 5/50 high power field） in all cases. The patients were disease-free for 10.5-42.2 mo after endoscopic enucleation.     

胃肠道间质瘤的临床病理分析和免疫组化特点

目的:探讨胃肠道间质瘤(GIST)的临床病理和免疫组化特点．方法:回顾性分析我院收治的24例GIST患者的临床病理及随访资料,并对其石蜡标本采用免疫组化SP染色法检测,分析其免疫组化特点．结果:本组GIST发病年龄29—75(平均54．5±13．5),男16例,女8例,主要发生在胃、小肠、直肠．并多以腹部隐痛不适、腹部肿物、上消化道出血为首发症状,全部患者进行积极外科治疗,肿瘤切除率100％,术后复发转移率为14．3％．大多数病例为手术切除后确诊．肿瘤大小从2-16 cm(平均6．6±3．9 cm)．基本病理组织形态为梭形细胞型,上皮细胞型较少．5例核分裂像>5／50HPF者其肿瘤直径均>5 cm,其中4例伴有囊性变、出血或坏死．4例核分裂像为2-5／50HPF者肿瘤直径在3-5 cm之间．免疫组化染色CD117,CD34,Vim为弥漫强阳性,阳性率分别为91．7％,62．5％和66．7％．S-100阴性, SMA呈灶状阳性．结论:GIST好发于中老年,缺乏特异的临床表现,早期诊断困难,外科手术仍是未转移的GIST的首要治疗方式,并且其肿瘤细胞形态多变,排列方式多样,免疫组化染色为CD117, CD34和Vim阳性,S-100阴性,SMA呈灶状阳性．