胃肠道间质瘤的治疗进展

胃肠道间质瘤(GIST)是消化道最常见的间叶组织源性肿瘤,其确诊主要依赖于组织学证据和免疫组化标志。由于酪氨酸激酶抑制剂伊马替尼的成功应用,GIST的治疗方法已由原先简单的手术切除发展为针对病情采取包括手术、内镜治疗、辅助治疗和新辅助治疗在内的个体化治疗策略。此文对近年来GIST的各种治疗进展作一综述。     

胃肠道间质瘤的治疗进展

胃肠道间质瘤(GIST)是胃肠道最常见的间叶源性肿瘤,占间叶源性肿瘤的18%和消化道肿瘤的1%,每年发病率为1/10万~2/10万。恶性GIST根治性切除术后5年复发率高达50%,转移性GIST既往中位生存期仅9个月。目前,对直径≤2 cm的上消化道GIST首选内镜超声(EUS)检查,直径2 cm的GIST因恶变程度高,首选手术切除,对于手术切除困难、风险高的病灶可选择酪氨酸激酶抑制剂辅助化疗和新辅助化疗。本文就GIST的治疗进展作一综述。     

胃肠道间质瘤的手术治疗进展

胃肠道间质瘤（GIST）是一类起源于胃肠道间叶组织的肿瘤，具有多向分化特征，是一种具有恶性潜能的黏膜下肿瘤，可起源于黏膜肌层或固有肌层，可腔内生长，亦可腔外生长。目前手术切除是GIST首选且唯一可能治愈的方法。由于GIST的生长部位和生长方式的不同，导致最佳手术切除方法亦不相同，包括外科开腹或腹腔镜治疗、内科内镜下治疗以及多镜联合治疗等手术方式。随着微创技术的不断发展和成熟，本着创伤最小、疗效最大的原则，对GIST的手术治疗方式值得进一步深入探讨。     

内镜下切除与外科手术治疗胃肠道间质瘤的疗效比较

胃肠道间质瘤(gastrointestinal stromal tumor,GIST)是一类起源于间质的具有恶性潜能的肿瘤,是胃肠道最常见的间叶性肿瘤.本研究回顾性分析内镜下切除与外科手术治疗GIST的近期与远期疗效. 一、对象与方法 1.病例来源:纳入2007年1月至2009年12月四川大学华西医院行全瘤切除术后经病理证实为GIST的患者54例.其中在内镜下切除肿瘤患者23例,为内镜治疗组;通过外科开腹行胃局部或胃大部切除术患者31例,为外科手术组.为减少操作者技术水平不同而带来的偏倚,2组手术操作者尽量选择同一医师或有同等经验的医师.     

胃肠道间质瘤的诊治新进展

胃肠道间质瘤（GIST）是胃肠道最常见的间叶源性肿瘤,原癌基因c—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是一个重要的决定性因素。手术完整切除仍然是其首选治疗,分子靶向治疗是进展期GIST治疗上的一次飞跃,本文对GIST的诊治新进展进行综述。     

超声内镜在胃肠道间质瘤诊断中的应用进展

胃肠道间质瘤(gastrointestinal stromal tumor, GIST)是具有恶性潜能的间质瘤,不同恶性风险的GIST选择不同的治疗策略,如监测随访、内镜治疗、手术切除或/和药物治疗。因此,GIST的早期诊断以及风险评估对选择合适的治疗方案十分重要。目前,随着内镜技术的广泛应用,超声内镜(endoscopic ultrasound, EUS)、超声内镜弹性成像(endoscopic elastic imaging, EUS-EG)、超声内镜谐波增强(contrast-enhanced harmonic endoscopic ultrasound, CH-EUS)、超声内镜引导下细针抽吸/活检(endoscopic ultrasound-guided fine-needle aspiration/biopsy, EUS-FNA/EUS-FNB)被应用于GIST的诊断以及风险评估。本文主要对以上检查方式在GIST中的应用进行阐述。     

内镜下胃部胃肠道间质瘤切除适应证之华西经验

胃肠道间质瘤（gastrointestinal stromal tumor，GIST）是一类起源于间质的具有恶性潜能的肿瘤，是胃肠道最常见的间叶性肿瘤。 一、背景 开腹手术完整切除肿瘤是目前治疗GIST的首选方案，通常因周围淋巴结转移很少见，无需常规性淋巴结清扫。     

胃黏膜下肿瘤微创治疗的进展

胃黏膜下肿瘤经常于胃镜检查中被发现,给诊断及治疗带来了挑战.最值得讨论的胃黏膜下肿瘤为间质瘤,因其为胃肠道最常见的间质性肿瘤.由于胃肠道间质瘤存在潜在恶性的可能,治疗局限胃间质瘤的主要目标是达到边缘阴性的切除,这是一种获得完全治疗的有效方法.微创治疗已经广泛应用在切除胃肠道黏膜下肿瘤方面.随着内镜技术的迅猛发展,内镜下切除为胃黏膜下肿瘤的治疗提供了不同的方案.有些专家还提出了内镜与腔镜的联合治疗.除此以外,机器人切除黏膜下肿瘤也成为可能.本文主要介绍微创治疗胃黏膜下肿瘤的进展.     

胃肠间质瘤的治疗进展

胃肠间质瘤(gastrointestinal stromal tumors,GIST)是一组独立起源于胃肠道间质干细胞的肿瘤;属于消化道间叶性肿瘤,由Mazur和Clark提出并命名,多呈CD117免疫组化染色阳性。GIST的治疗主要依赖于早期发现和争取手术切除,但85%的患者术后会复发;不能手术者和已有转移者对常规的     

胃间质瘤治疗方法的研究进展

胃肠道间质瘤(GIST)是一类常见的消化道间叶性肿瘤,普遍认为它具有潜在恶性倾向,较少出现淋巴结转移。目前手术切除是治疗胃间质瘤的金标准,可以达到完全切除,但创伤较大。随着内镜技术的不断发展,对于早期发现的直径较小的胃间质瘤,内镜下治疗具有创伤小、疗效显著等特点。新型靶向药物的研发促进了胃间质瘤治疗的进展,为治疗提供了更多的选择。该文主要就胃间质瘤治疗方法的研究进展作一综述。     

Endoscopic Resection of Small Gastrointestinal Stromal Tumors

Background  

The common treatment principle of gastrointestinal stromal tumors (GISTs) is complete resection of the tumor. The rapid development of endoscopic skill makes it possible to resect GISTs en-bloc through the endoscopic approach.     

Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome

This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. Recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.     

Gastrointestinal stromal tumors: the role of the gastroenterologist in diagnosis and risk stratification

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that are best classified as sarcomas with variable aggressiveness. They are typically treated with surgical resection and adjuvant tyrosine kinase inhibitors or, for advanced/metastatic GISTs, with tyrosine kinase inhibitors alone. Gastroenterologists are often the first to detect GISTs and are, therefore, tasked with diagnosis and facilitation of early intervention. Diagnostic tools including various imaging techniques such as endoscopic ultrasound (EUS) and EUS-assisted tissue sampling are critical for an accurate diagnosis. In the case of small tumors, EUS-assisted resection or ligation techniques to treat asymptomatic small tumors have been described. This paper reviews current evidence for the diagnosis and management of GISTs, with an emphasis on the role of the gastroenterologist.     

Current treatment strategies and future perspectives for gastrointestinal stromal tumors

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from the gastrointestinal tract, mostly from the stomach. GISTs are derived from the myenteric interstitial cells of Cajal and are caused by several mutations in the c-kit and platelet-derived growth factor receptor genes. Clinically, GISTs are detected by endoscopic and imaging findings and are diagnosed by immunostaining. Surgery is the first line of treatment, and if the tumor is relatively small, minimally invasive surgery such as laparoscopy is performed. In recent years, neoadjuvant therapy has been administered to patients with GISTs that are suspected of having a large size or infiltration to other organs. Postoperative adjuvant imatinib is the standard therapy for high-risk GISTs. It is important to assess the risk of recurrence after GIST resection. However, the effect of tyrosine kinase inhibitor use will vary by the mutation of c-kit genes and the site of mutation. Furthermore, information regarding gene mutation is indispensable when considering the treatment policy for recurrent GISTs. This article reviews the clinicopathological characteristics of GISTs along with the minimally invasive and multidisciplinary treatment options available for these tumors. The future perspectives for diagnostic and treatment approaches for these tumors have also been discussed.     

Therapeutic procedures for submucosal tumors in the gastrointestinal tract

This review is part three of three and will present an update on the therapeutic options and procedures concerning gastrointestinal （GI） submucosal tumors （SMTs）. The aim of this paper is to investigate the treatments of GI SMTs and to present a case of a gastrointestinal stromal tumor （GIST）. Literature searches were performed to find information on therapy for GI SMTs. Based on these searches, the optimal therapeutic procedures could be outlined. The choice of treatment of localized tumors is endoscopic resection if possible or, alternatively, laparoscopic resection or surgical resection by an open procedure. However, benign SMTs should only be excised if symptoms are present, and GISTs should be treated with particular precautions. Irresectable or recurrent GISTs may be successfully treated with the tyrosine kinase inhibitor, imatinib.     

Endoscopic treatment for gastrointestinal stromal tumor: Advantages and hurdles

One of the most prominent characteristics of gastrointestinal stromal tumors(GISTs) is their unpredictable and variable behavior. GISTs are not classified as "benign" or "malignant" but are rather stratified by their associated clinical risk of malignancy as determined by tumor size, location, and number of mitoses identified during surgicalhistology. The difficulty in assessing the malignant potential and prognoses of GISTs as well as the increasing incidence of "incidental GISTs" presents challenges to gastroenterologists. Recently, endoscopic enucleation has been actively performed as both a diagnostic and therapeutic intervention for GISTs. Endoscopic enucleation has several advantages, including keeping the stomach intact after the removal of GISTs, a relatively short hospital stay, a conscious sedation procedure, relatively low cost, and fewer human resources required compared with surgery. However, a low complete resection rate and the risk of perforation could reduce the overall advantages of this procedure. Endoscopic full-thickness resection appears to achieve a very high R0 resection rate. However, this technique absolutely requires a very skilled operator. Moreover, there is a risk of peritoneal seeding due to large active perforation. Laparoscopy endoscopy collaborations have been applied for more stable and pathologically acceptable management. These collaborative procedures have produced excellent outcomes. Many procedures have been developed and attempted because they were technically possible. However, we should first consider the theoretical basis for each technique. Until the efficacy and safety of sole endoscopic access are proved, the laparoscopy endoscopy collaborative procedure appears to be an appropriate method for minimally destructive GIST surgery.     

Endoscopic ultrasound-guided fine-needle aspiration for diagnosing a rare extraluminal duodenal gastrointestinal tumor

Duodenal gastrointestinal stromal tumors(GISTs) are extremely rare disease entities, and the extraluminal type is difficult to diagnose. These tumors have been misdiagnosed as pancreatic tumors; hence, pancreaticoduodenectomy has been performed, although partial duodenectomy can be performed if accurately diagnosed. Developing a diagnostic methodology including endoscopic ultrasonography(EUS) and fine-needle aspiration(FNA) has allowed us to diagnose the tumor directly through the duodenum. Here, we present a case of a 50-year-old woman with a 27-mm diameter tumor in the pancreatic uncus on computed tomography scan. EUS showed a well-defined hypoechoic mass in the pancreatic uncus that connected to the duodenal proper muscular layer and was followed by endoscopic ultrasoundguided fine-needle aspiration(EUS-FNA). Histological examination showed spindle-shaped tumor cells positively stained for c-kit. Based on these findings, the tumor was finally diagnosed as a duodenal GIST of the extraluminal type, and the patient underwent successful mass resection with partial resection of the duodenum. This case suggests that EUS and EUS-FNA are effective for diagnosing the extraluminal type of duodenal GISTs, which is difficult to differentiate from pancreatic head tumor, and for performing the correct surgical procedure.     

Is the invasive approach for all the upper gastrointestinal mesenchymal tumors necessary?]

BACKGROUND/AIMS: Mesenchymal tumors are the most frequent submucosal tumors in gastrointestinal trail. We reviewed the mesenchymal tumors which are confirmed by pathology to examine whether the invasive approach of all mesenchymal tumors is necessary. METHODS: This study was performed on fifty-nine patients who has mesenchymal tumors confirmed by endoscopic or surgical resection from January 2000 to June 2004. RESULTS: Mesenchymal tumors consisted of thirty-six gastrointestinal stromal tumors (GISTs), 20 leiomyomas and 3 schwannomas. All the esophageal tumors were leiomyoma (12/12, 100%). In stomach, there were 32 GISTs (76.2%), 7 leiomyomas (16.7%) and 3 schwannomas (7.1%). And there were 4 GISTs (80.0%) and 1 leiomyoma (20.0%) in duodenum. Tumors less than 1 cm in maximal diameter were leiomyoma or GISTs with very low risk of aggressive behavior. 56.1% of the tumors larger than 1 cm consisted of low, intermediate or high risk GISTs. CONCLUSIONS: Biopsy must be considered according to its size and anatomic location of mesenchymal tumors. The invasive approach for every esophageal submucosal tumor is not necessary unless the size is very large, because most of them are benign in nature. However, the gastric submucosal tumor with more than 1 cm in diameter should be carefully and regularly followed up or biopsied because it cannot be assumed to be benign for any GIST more than 1 cm in size at the present time, safely.     

Gastrointestinal stromal tumors: review on morphology, molecular pathology, diagnostics, prognosis and treatment options

Gastrointestinal stromal tumors (GISTs) are the most common non-epithelial mesenchymal tumors of the gastrointestinal tract. GISTs represent a specific group of mesenchymal tumors with uncertain biological behaviors. These tumors are assumed to originate from progenitor cells, usually unable to self-regenerate, which differentiate towards Cajal cells. Apart from common GISTs that occur predominantly in adulthood, a heterogeneous group of tumors has been described that are morphologically identical with GIST, but have a specific clinical presentation and biological properties. Approximately 30% of newly diagnosed GISTs are malignant or have a high potential for malignancy. Currently, GISTs are routinely identified with histological, immunohistochemical, and molecular genetic assays. However, clinical diagnoses, particularly of small or intramural GISTs, might be difficult. The most useful techniques for imaging and monitoring disease progression are endoscopic examinations and fused PET/CT imaging. Surgical treatment is the first-line treatment and the only method that might lead to full remission in patients with a primary GIST. There is currently no consensus on the issues of whether to perform resections in patients with positive margins or resections of metastases. Endoscopic resection could represent a relatively simple and less aggressive alternative as compared to traditional surgery in the treatment of small sized GISTs. Biological therapy with imatinib mesylate is recommended for patients with newly diagnosed, locally advanced, inoperable, or metastasizing gastrointestinal GISTs that express the c-KIT protein. Treatment may reduce a primary tumor to a size small enough for surgical excision. Current research is focusing on the development of new therapies for the treatment of advanced disease and/or disease prophylaxis.     

Laparoscopic endoscopic cooperative surgery as a minimally invasive treatment for gastric submucosal tumor

Laparoscopic wedge resection is a useful procedure for treating patients with submucosal tumor (SMT) including gastrointestinal stromal tumor (GIST) of the stomach. However, resection of intragastric-type SMTs can be problematic due to the difficulty in accurately judging the location of endoluminal tumor growth, and often excessive amounts of healthy mucosa are removed; thus, full-thickness local excision using laparoscopic and endoscopic cooperative surgery (LECS) is a promising procedure for these cases. Our experience with LECS has confirmed this procedure to be a safe, feasible, and minimally invasive treatment method for gastric GISTs less than 5 cm in diameter, with outcomes similar to conventional laparoscopic wedge resection. The important advantage of LECS is the reduction in the resected area of the gastric wall compared to that in conventional laparoscopic wedge resection using a linear stapler. Early gastric cancer fits the criteria for endoscopic resection; however, if performing endoscopic submucosal dissection is difficult, the LECS procedure might be a good alternative. In the future, LECS is also likely to be indicated for duodenal tumors, as well as gastric tumors. Furthermore, developments in endoscopic and laparoscopic technology have generated various modified LECS techniques, leading to even less invasive surgery.