胃肠道间质瘤的临床诊断

胃肠道问质瘤(gastrointestinal stromal tumors,GIST)是胃肠道最常见的间叶源性肿瘤.过去由于病理手段的限制,多将其诊断为平滑肌源性或神经源性肿瘤.近年来随着病理学的进展发现GIST具有自身形态学、免疫表型和遗传学特征.本文综合近年来的研究,结合复习文献,对GIST的临床诊断特征进行相关讨论.     

Stathmin在胃肠道间质瘤组织中的表达与临床病理因素之间的关系

[目的]分析原发性胃肠道间质瘤(GIST)中微管解聚蛋白(Stathmin)的表达程度与临床病理因素之间的相关性,探讨Stathmin是否是GIST患者的诊断、治疗及其预后中发挥重要作用的蛋白。[方法]采用免疫组化学技术检测96例GIST组织中Stathmin蛋白的表达,采用SPSS25.0软件统计分析Stathmin蛋白的表达水平与患者一般临床病理学特征之间的相关性。[结果]Stathmin在GIST组织中阳性率为45.8%(44/96),在GIST组织中高表达;Stathmin阳性表达与患者肿瘤危险度、CD34有统计学意义(P0.05、P0.01),与患者性别、年龄、肿瘤部位、肿块大小、浸润深度等差异无统计学意义(P0.05)。[结论]在GIST患者中Stathmin高表达与肿瘤危险度分级、CD34(+)密切相关。Stathmin有可能成为判别该病的辅助诊断及患者病情恶化的指标,为今后GIST患者的诊断、临床治疗策略及预后判断提供理论依据。     

胃肠道间质瘤的诊断和治疗

编者按胃肠道间质瘤(gastrointestinal stromal tumors,GIST)是一类独立来源于胃肠道间叶组织、以梭形细胞为主非定向分化的肿瘤,因其多发生于空腔脏器的肌层,曾被误认为平滑肌瘤、上皮平滑肌肉瘤等.近几年,我们对GIST的认识从各个方面都有了很大的提高.GIST的影像学诊断是目前诊断GIST的主要方法,其影像学特征对判定肿瘤生物学行为,了解肿瘤周围组织浸润情况及有无远处转移提供一定的信息;对于疾病的早期发现、及时治疗、治疗方案的选择以及预后有着重要意义.在GIST的病理研究方面取得了不少进展,为GIST诊断和危险度判定提供了重要依据,现如今,GIST的综合治疗已经越来越受到人们的重视,相信随着研究的不断深入,会有更多更好的办法应用于GIST的治疗,我们对此充满信心.山西医科大学第一医院放射科李健丁教授特组织本期焦点论坛,将围绕GIST的影像诊断、病理诊断、临床治疗问题进行初步讨论,希望能引起同行专家的兴趣,提出更深入的见解.     

DOG1蛋白在胃肠道间质瘤中的表达及相关性分析

目的 分析DOG1蛋白与胃肠道间质瘤(GIST)的相关性.方法 收集2005年1月至2010年6月77例GIST患者,应用免疫组化法检测GIST中DOG1蛋白的表达,并结合原有免疫组化指标CD117、CD34的检测结果进行对照分析.结果 77例GIST患者中,DOG1蛋白和CD117的阳性表达率分别为96.1%(74/77)和90.9%(70/77),两组间差异无统计学意义(P＞0.05).不同性别、年龄组、病变部位及危险度间DOG1蛋白阳性表达率差异亦无统计学意义(P值均＞0.05).7例CD117阴性表达的GIST患者中6例DOG1蛋白阳性表达.3例DOG1蛋白阴性表达的GIST中2例CD117阳性表达.结论 DOG1蛋白作为一种新的GIST标志物,具有与CD117同样的高敏感性,与CD117联用于GIST诊断互补性较好,尤其对CD117阴性的GlST诊断价值优势较大,但不能作为划分GIST危险度的指标.     

MRI在诊断及评估胃肠道间质瘤侵袭危险度中的应用价值分析

目的分析MRI在诊断及评估胃肠道间质瘤(GIST)侵袭危险度中的应用价值。方法选择我院2012年5月至2014年3月已行MRI检查并经手术病理证实的30例胃肠道间质瘤患者为研究对象,将其MRI检查结果与病理结果进行对比。同时观察和分析不同侵袭危险度(低危、中危、高危)患者MRI表现征象(形态、边界、信号等)特点,并进一步分析MRI征象与不同侵袭危险度的相关性。结果 30例患者中低危12例,中危7例,高危11例。高危组与低危组比较,形态、边界、信号均匀性及ADC值差异均有统计学意义(P0.05)。高危组ADC值明显高于中危组(P0.05)。低危组与高危组比较,边界、信号均匀性差异有统计学意义(P0.05)。结论 MRI扫描与ADC值能协助GIST术前侵袭危险度评估。     

丝切蛋白1在胃肠道间质瘤组织中的表达及其临床意义

[目的]探究丝切蛋白1(cofilin-1)在胃肠道间质瘤(GIST)组织中的表达水平与临床指标间的相关性。[方法]选取96例GIST患者术后病理切片组织进行免疫组化染色,检测其中cofilin-1的表达水平,结合临床数据及病理结果,分析cofilin-1与GIST组织临床指标间的相关性。[结果]96例GIST患者年龄、性别及肿瘤发生部位与cofilin-1的表达均无显著相关(P0.05);GIST患者肿瘤浸润深度、肿瘤直径、病理性核分裂象及其危险度分级与cofilin-1的表达呈正相关(P0.05,r0);GIST患者肿瘤浸润深度、肿瘤直径、病理性核分裂象及其危险度分级均为cofilin-1表达的危险因素,差异均有统计学意义(P0.05,OR1),其中肿瘤浸润深度及其危险度分级皆为cofilin-1表达的独立危险因素,差异均有统计学意义(P0.05,OR1)。[结论]cofilin-1的表达与GIST的浸润深度、肿瘤大小、病理性核分裂象及其危险度分级呈正相关,其中肿瘤浸润深度及其危险度分级为独立危险因素,cofilin-1有望成为预示GIST的发生、进展、转移以及评估其预后的生物标志物以及崭新的诊疗靶标。     

影像学检查在胃肠道间质瘤诊断与疗效评估中的应用比较

胃肠道间质瘤(GIST)是胃肠道最常见的间叶性肿瘤,近年来由于影像学和病理学研究的进展,人们对其认识也不断加深,各种影像学检查对GIST术前诊断的报道不断增多.此文将对影像学检查在GIST诊断及疗效评估中的应用作一简要阐述,探讨GIST诊断的现状和各种影像学检查的优缺点,为临床选择何种影像学检查提供参考.     

胃肠道间质瘤预后研究进展

胃肠道间质瘤(GIST)是人类胃肠道最常见的间叶源性肿瘤,大多数GIST有原癌基因c-kit突变.近年的研究发现,在无c-kit突变的胃肠道间质瘤中,存在血小板源性生长因子受体-α(PDGFR-α)基因的突变.此外,尚存在很多基因和蛋白参与了GIST的发生和发展.与一般胃肠道肿瘤相比,GIST在组织发生、临床病理、诊断治疗、预后等方面有着不同的特点.本文主要综述有关GIST预后的相关指标的研究进展.     

36例胃肠道间质瘤的临床诊治

目的 总结胃肠道间质瘤（GIST）的临床诊疗经验和病理学特征.方法 回顾性分析1995年1月至2005年4月收治的36例GIST的临床和病理资料.结果 最常见临床表现为腹部隐痛不适19例,消化道出血12例,腹部包块7例.最常见病变部位为胃26例,小肠8例.病程1天～2年.肿块平均直径7.2 cm.CD117阳性29例,CD34阳性30例.总的随访率为31/36（86.1%）,11例死亡.结论 GIST术前确诊较困难,确诊主要依靠病理学观察和免疫组化检测,联合CD117和CD34检测可提高GIST诊断.手术完整切除肿瘤是治疗GIST的关键.     

胃肠道间质瘤的临床特征及治疗进展

胃肠道间质瘤(gastrointestinal stromal tumor,GIST)是胃肠道最常见的间叶性肿瘤,近年来随着分子生物学、组织病理学和临床医学的研究进展,对其认识不断加深.传统手术与分子靶向药物相结合对GIST的治疗显得尤为重要,以伊马替尼和舒尼替尼等药物为代表的多靶点和多激酶抑制剂在治疗不可切除及复发性GIST中更是受到高度关注.本文主要对该病的基因分析、病理组化特点、手术及辅助治疗相关进展作一综述.     

LONG‐TERM FOLLOW UP OF PATIENTS WITH SMALL GASTROINTESTINAL STROMAL TUMORS IN THE STOMACH USING ENDOSCOPIC ULTRASONOGRAPHY‐GUIDED FINE‐NEEDLE ASPIRATION BIOPSY

Background: Gastrointestinal stromal tumors (GIST) are one of the most common mesenchymal tumors of the gastrointestinal tract. GIST are defined by positive immunohistochemical staining for KIT or CD34 and thus are generally diagnosed after surgery. Because small GIST are rarely diagnosed before surgery, the clinical course of these small tumors is not clear. The aim of the present study was to follow changes in size and configuration of small GIST that were pathologically confirmed using endoscopic ultrasonography‐guided fine‐needle aspiration biopsy (EUS‐FNAB). Methods: Between July 1997 and December 2003, 16 tumors in 16 patients (10 men and 6 women) with an immunohistochemical diagnosis of GIST were regularly followed in our hospital. The median patient age when EUS‐FNAB was performed was 62 years (range 26–82 years) and the median follow‐up period was 4.9 years (range 0.5–9.6 years). Results: Fourteen tumors showed no remarkable changes in size and shape during follow up compared with the initial diagnosis. Two tumors enlarged: one tumor approximately doubled its diameter in 8 years and the other tumor increased from 1.8 cm at diagnosis to up to 10 cm after only 2 years. Doubling time of the latter tumor was calculated as 3.1 months. Conclusions: We conclude that EUS‐FNAB might be a good modality for final diagnosis of GIST without surgery, and that GIST without rapid growth on follow up can be endoscopically followed.     

Clinical implications of doubling time of gastrointestinal submucosal tumors

AIM To evaluate the efficacy of doubling time(DT) of gastrointestinal submucosal tumors(GIST).METHODS From April 1987 through November 2012, a total of 323 patients were given a final histopathological diagnosis of GISTs on surgical resection or endoscopic ultrasound-guided fine-needle aspiration(EUS-FNA) in Kitasato University East Hospital or Kitasato University Hospital. We studied 53 of these patients(34 with resected tumors and 19 with unresected tumors) whose tumors could be measured on EUS on at least two successive occasions. The histopathological diagnosis was GIST in 34 patients, leiomyoma in 5, schwannoma in 3, ectopic pancreas in 1, hamartoma in 1, cyst in 1, Brunner's adenoma in 1, and spindle-cell tumor in 7. We retrospectively calculated the DT of GISTs on the basis of the time course of EUS findings to estimate the growth rate of such tumors.RESULTS The DT was 17.2 mo for GIST, as compared with 231.2 mo for leiomyoma, 104.7 mo for schwannoma, 274.9mo for ectopic pancreas, 61.2 mo for hamartoma, 49.0 mo for cyst, and 134.7 mo for Brunner's adenoma. The GISTs were divided into risk classes on the basis of tumor diameters and mitotic figures(Fletcher's classification). The classification was extremely low risk or low risk in 28 patients, intermediate risk in 3, and high risk in 3. DT of GIST according to risk was 24.0 mo for extremely low-risk plus low-risk GIST, 17.1 mo for intermediate-risk GIST, and 3.9 mo for high-risk GIST. DT of GIST was significantly shorter than that of leiomyoma plus schwannoma(P 0.05), and DT of high-risk GIST was significantly shorter than that of extremely low-risk plus low-risk GIST(P 0.05).CONCLUSION For GIST, a higher risk grade was associated with a significantly shorter DT. Small SMTs should initially be followed up within 6 mo after detection.     

胃肠道间质瘤的诊断治疗

胃肠道间质瘤(gastrointestinal stromal tumors,GIST)是消化系最常见的间叶组织源性肿瘤,是一种潜在恶性的肿瘤.其临床表现缺乏特异性,术前诊断上存在较大困难.近几年其发病机制已经逐渐被人们所认识,诊断及治疗水平上也有了很大的提高.原癌基因kit突变是其主要发病机制之一.以CD117为代表的免疫织化学染色在其诊断中作为一个重要的决定性因素.治疗上目前注重于综合治疗,手术完整切除仍然是其首选治疗,包括新辅助治疗及术后辅助治疗在内的分子靶向治疗的出现成为GIST治疗上的一次巨大进步,甲磺酸伊马替尼等选择性酪氨酸激酶抑制剂制剂的出现,给GIST患者的治疗带来了新的希望.     

CT和超声内镜诊断胃肠道间质瘤的现状

胃肠道间质瘤是原发于胃肠道和腹部的间叶源性肿瘤,绝大多数存在c-Kit基因突变。内镜检查胃肠道间质瘤有一定困难,而CT和超声内镜结合有助于胃肠道间质瘤的定位和良、恶性的判断,对指导临床治疗和估计预后有一定的价值。     

Current clinical management of gastrointestinal stromal tumor

Gastrointestinal stromal tumors(GISTs) are the most common malignant subepithelial lesions(SELs) of the gastrointestinal tract. They originate from the interstitial cells of Cajal located within the muscle layer and are characterized by over-expression of the tyrosine kinase receptor KIT. Pathologically, diagnosis of a GIST relies on morphology and immunohistochemistry [KIT and/or discovered on gastrointestinal stromal tumor 1(DOG1) is generally positive]. The prognosis of this disease is associated with the tumor size and mitotic index. The standard treatment of a GIST without metastasis is surgical resection. A GIST with metastasis is usually only treated by tyrosine kinase inhibitors without radical cure; thus, early diagnosis is the only way to improve its prognosis. However, a GIST is usually detected as a SEL during endoscopy, and many benign and malignant conditions may manifest as SELs. Conventional endoscopic biopsy is difficult for tumors without ulceration. Most SELs have therefore been managed without a histological diagnosis. However, a favorable prognosis of a GIST is associated with early histological diagnosis and R0 resection. Endoscopic ultrasonography(EUS) and EUS-guided fine needle aspiration(EUSFNA) are critical for an accurate diagnosis of SELs. EUSFNA is safe and effective in enabling an early histological diagnosis and adequate treatment. This review outlines the current evidence for the diagnosis and management of GISTs, with an emphasis on early management of small SELs.     

胃肠道间质瘤的影像学研究进展

胃肠道间质瘤(gastrointestinal stromal tumors, GIST)是胃肠道最常见的非上皮性肿瘤,近年随着影像学的发展,诊断胃肠道间质瘤的报道有逐年增高的趋势.本文综述了近年来关于GIST影像诊断的研究进展.     

Intra-abdominal desmoid tumors mimicking gastrointestinal stromal tumors——8 cases: A case report

BACKGROUND Intra-abdominal desmoid tumors(DTs) can mimic recurrence or progression of gastrointestinal stromal tumors(GISTs). Differential diagnosis is important to avoid unnecessary or inappropriate treatment.CASE SUMMARY All 8 patients experienced surgical resection of GIST, and median time to diagnosis of DT was 1.8 years after surgical resection. All sites of DT were in the peritoneum around the surgical sites of GIST. The following clinical suspicion coupled with radiological findings contributed to the suspicion of intraabdominal DTs:(1) Occurrence of a new single lesion in the peritoneum around the surgical sites of GIST;(2) uncontrolled lesion with imatinib while other lesions being controlled with imatinib;(3) well-defined ovoid shaped lesion with delayed or mild enhancement and absence of necrosis, hemorrhage, and cystic change on computed tomography; and(4) a lesion showing mild or no hypermetabolic activity on 18 fluorodeoxyglucose-positron emission tomography,contrary to initially hyperactive lesion of GIST. All DTs were surgically removed except for one unresectable DT and only one DT recurred at another site of peritoneum, which was also surgically removed.CONCLUSION Intra-abdominal DT should be a differential diagnosis for a new single lesion in patients with GIST.     

胃肠道外间质瘤的影像诊断新进展

自1983年Mazur和Clark提出胃肠道间质瘤的概念以来,目前已成为病理学和临床研究的热点.发生于胃肠道外的间质瘤(extra-gastrointestinal stromal tumors,EGIST)比较少见,其影像学表现在诊断中起着重要的作用.本文就EGIST的影像诊断现状作一综述.     

Thoracoscopic enucleation for small-sized gastrointestinal stromal tumor of the esophagus: report of two cases

Gastrointestinal stromal tumors (GISTs) rarely occur in the esophagus. Surgical approaches for such tumors have not been established, since the standard wedge or segmental resection that is used for intra-abdominal GIST is not possible in the esophagus. We report two cases of small esophageal GIST in which thoracoscopic enucleation was performed. Both patients underwent the thoracoscopic surgery using four trocars. The tumor size was 43 and 32 mm in patients 1 and 2, respectively. The operating time was 240 and 238 min. The final diagnosis was as low-risk GIST in both patients. Postoperative course was uneventful and both patients have been disease-free at a follow-up of 40 and 32 months. Considering the special case of the esophagus and the very good prognosis of low-risk tumors, enucleation under the thoracoscopic technique may be feasible for small-sized esophageal GIST as a minimally invasive surgery. We also review the literature in this report.