胃肠管间质瘤生物学行为相关研究新进展

胃肠管间质瘤是发生于消化管的最常见的间叶组织来源肿瘤,其生物学行为的预测成为现今研究的一大热点.本文就近年来胃肠管间质瘤生物学行为相关研究的进展情况进行综述.     

十二指肠胃肠间质瘤22例诊治分析

目的探讨十二指肠胃肠间质瘤的临床特征、诊断与治疗。方法回顾分析2005年1月至2010年9月诊治的22例十二指肠胃肠间质瘤患者的临床病例资料。本组患者均行手术切除治疗,包括肿瘤局部切除12例,其中1例合并远端胃大部切除及右肾切除;十二指肠节段切除6例;胰十二指肠切除术4例,其中1例合并右半结肠切除。结果危险度分级为极低危组4例,低危组5例,中危组8例,高危组5例。CD117阳性率95．5％（21／22）,CD34阳性率86．4％（19／22）,SMA阳性率54．5％（12／22）,S-100阳性率9．1％（2／22）。术后随访10—66个月,平均随访30．9个月,其中20例获得完整随访,2例死于肿瘤复发或转移,余均无瘤生存。结论内镜超声和CT是术前诊断十二指肠胃肠间质瘤的有效方法,确诊需依靠病理及免疫组化。外科手术仍是当前十二指肠胃肠间质瘤最主要的治疗方法,需依据肿瘤的大小及部位采取不同的手术方式。高危患者术后联合靶向治疗可改善预后。     

Gastrointestinale Stromatumoren (GIST)

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract. GIST occur predominantly in the stomach and less frequently in the extraduodenal small bowel, the colon, and the rectum; rarely, GIST can be found in the esophagus and the duodenum. Due to their biological behavior, the primary treatment goal for localized primary GIST is complete resection, without the need for lymphadenectomy or wide resection margins. Thus, gastric wedge resections and segmental resections of the small bowel are the most common surgical procedures for treating primary GIST. Surgical therapy of extensive primary tumors or of metastatic or recurrent GIST should be integrated into a multimodal therapeutic concept that includes targeted therapy with tyrosine kinase inhibitors, such as imatinib.     

Cutaneous metastases from a gastrointestinal stromal tumor of the stomach: review of literature

Gastrointestinal stromal tumors (GIST) are neoplasms of mesenchymal origin which may or may not be malignant. Malignant GIST rarely metastasize to distal organs. A case of cutaneous metastasis from GIST has been presented. This case is particularly interesting because not only did the patient have multiple metastases from GIST, but eventually developed cutaneous metastasis. This phenomenon has not been reported in the literature. The most current literature on gastrointestinal stromal tumors has been reviewed.     

Surgery on gastrountestinal stromal tumor CD117+ (G.I.S.T.): personal experience

INTRODUCTION: Gastrointestinal stromal tumors are the most frequent (0,5-3%) mesenchymal tumors in the gastrointestinal tract. They probably originate from the interstitial cells of Cajal and are characterized by an anomaly of c-kit receptor, for a stem growth factor, with tyrosine-kinase activity (c-kit). This mutation causes a permanent activation of the receptor and uncontrolled cell growth. These tumors are associated with low survival in cases of advanced or metastatic disease. Imatinib, a tyrosine kinase inhibitor, induces improved survival in these patients. CASE REPORTS: The authors discuss two cases of gastrointestinal stromal tumors surgically treated and also review the pathophysiology, diagnosis difficulties, role of surgery today, and treatment-related outcome of this type of tumors.     

胃肠间质瘤预后相关因素

胃肠间质瘤（GIST）是一组起源于胃肠道间叶源性的肿瘤,其发病率低,恶性程度有时难以评估。即使完整切除GIST后,部分病人术后仍容易发生肿瘤复发或远处转移,准确的评估GIST恶性程度及预后影响因素对指导临床个体化治疗有着重要的意义。近年来,随着临床医师对其认识的不断加深,根据其预后因素制定了多种复发危险度分级标准以判断GIST预后的评估模型。GIST预后的影响因素有肿瘤部位、大小、核分裂像数、肿瘤是否破裂、基因突变类型以及肿瘤免疫组化标记物等。     

Extragastrointestinal stromal tumor (EGIST)--a case review

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. Due to the presence of thyrosine kinase receptors within the tumor tissue, GIST is thought to originate from gastrointestinal pacemaker cells, the intersticial cells of Cajal. Tumors with the same morphological and imunohistochemical characteristics detected outside the gastrointestinal tract, are called extragastrointestinal stromal tumors (EGIST). Biological characteristics of these tumors is uncertain and the malignancy rates are difficult to predict. Surgical R0 resection in resecable tumors is the only option with the potential for complete cure. Nevertheless, the recurrence rates are high. Adjuvant biological treatment with imatinib, a thyrosine kinase inhibitor, reduces the risk of relapses. Imatinib administration is also the principal treatment method in metastatic GIST disorders. The article offers a short and complex overview of gastrointestinal stromal tumor (GIST) problematics and presents a case report of a patient suffering from EGIST of mesocolon transversum treated by R0 resection which was performed under multidisciplinary cooperation, with a specialist follow up.     

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胃肠间质瘤是近年重新认识并且在治疗上取得较大突破的实体肿瘤类型。从发现其主要的发病机制开始,不久即出现了针对性的治疗药物,成为最早进入"靶向治疗"的肿瘤类型之一,体现了转化医学倡导的从实验室研究成果到临床应用的快速转化。回顾10余年来对该病的认识与诊治过程,本身就是对于肿瘤转化医学这一新兴的医学研究模式极佳的诠释。     

Synchronous gastrointestinal stromal tumor (GIST) and multiple digestive neoplasms, benign and malignant

Gastrointestinal stromal tumors (GIST) are a broad category of mesenchymal, non-epithelial primary tumors of the digestive tract, located in the wall of hollow viscera, from the esophagus to the anus and often in adjacent mesentery and omentum. They are clinically unpredictable (may be discovered incidentally during an imagistic investigation or during surgery for other pathological entity, or at necropsy) and also have an unpredictable behavior (GISTs with very low risk, with low or moderate malignancy, which have benign histopathologic features but can recurr or can metastasize). The case we present here represents a rare association between a synchronous gastrointestinal stromal tumor (GIST) and multiple gastric benign and malignant tumors.     

Risk factors for malignant evolution of gastrointestinal stromal tumors

Gastrointestinal stromal tumors are the most frequent non-epithelial digestive tumors, being classified in the group of primitive mesenchymal tumors of the digestive tract. These tumors have a non predictable evolution and where stratified regarding the risk for malignant behavior in 4 categories: very low risk, low risk, intermediate risk and high risk. We performed a retrospective non randomised study including the patients with gastrointestinal stromal tumors treated in the Department of General Surgery and Liver Transplantation of Fundeni Clinical Institute in the period January 2002 - June 2007, to define the epidemiological, clinico-paraclinical, histological and especially evolutive features of the gastrointestinal stromal tumors from this group, with a special regard to the risk factors for their malignant behavior. The most important risk factors in gastrointestinal stromal tumors are the tumor size and the mitotic index, based on them being realised the classification of Fletcher in the 4 risk categories mentioned above. In our group all the local advanced or metastatic gastrointestinal stromal tumors, regardless of their location, were classified in the group of high risk for the malignant behavior. The gastric location and the epithelioid type were positive prognostic factors, and the complete resection of the tumor, an other important positive prognostic feature, was possible in about 80% of the cases, probably because the gastrointestinal stromal tumors in our study were diagnosed in less advanced evolutive situations, only about one third being metastatic and about 14% being locally advanced at the time of diagnose. The association with other neoplasias was in our cases insignificant, only 5% of the patients presenting concomitant malignant digestive tumors and 7.6% intraabdominal benign tumors. Gastrointestinal stromal tumors remain a challenge for the medical staff, regarding their diagnose and therapeutical management, the stratification of the risk for their malignant behavior being essential for the evolution of these patients.     

Gastrointestinal stromal tumors in patients with neurofibromatosis 1: a clinicopathologic and molecular genetic study of 45 cases

Gastrointestinal stromal tumors (GISTs), the specific KIT- or PDFGRA-signaling driven mesenchymal tumors, most commonly occur sporadically, but there seems to be some increased tendency for these tumors to develop in patients with neurofibromatosis 1 (NF1). The clinicopathologic profile, KIT, and PDGFRA mutation status and long-term prognosis of patients with GIST in NF1 are incompletely characterized. In this study, we analyzed 45 patients who had NF1 and GIST. There were 26 females and 19 males with a median age of 49 years (10 years lower than the median age of GIST patients in general). A great majority of tumors occurred in the jejunum or ileum, with multiple tumors occurring in 28 cases. Ten patients had a duodenal and one had a gastric GIST. The most common presentations were gastrointestinal bleeding and anemia, and many patients had intermittent bleeding over several years. The majority of the tumors were small and mitotically inactive; only 7 had mitotic activity >5/50 HPFs and 15 tumors were >5 cm. Associated Cajal cell hyperplasia was common. One patient had an intraabdominal peri-intestinal neurofibroma. Five of 35 patients with follow-up died of metastatic disease; all of these had a tumor >5 cm, mitotic rate >5/50 HPFs, or both; three of these tumors were located in the duodenum. The presence of multiple small tumors was not associated with progressive disease. Most patients with long-term follow-up enjoyed a good prognosis; 2 died of other NF1-associated tumors (malignant peripheral nerve sheath tumors, brain tumor). None of the 16 tumors from 15 patients had a KIT exon 9, 11, 13, or 17 or PDGFRA exon 12 or 18 mutation as is typically seen in sporadic GISTs, indicating that GISTs in NF1 patients have a different pathogenesis than sporadic GISTs.     

Giant GIST of the mesocolon: report of a case

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal cells of the gastrointestinal tract, that strongly express a class III receptor tyrosine kinase, called KIT, due to some mutations in the KIT proto-oncogene. Two thirds of GISTs are found in the stomach, 20% to 50% in the small bowel (one third in the duodenum), and 5% to 15% in colon and rectum; GISTs, however, may rarely be found also in the oesophagus, omentum, mesentery or the retroperitoneum. Their treatment is strictly surgical, and only R0 resection can achieve good RESULTS: Treatment with Imatinib seems to be promising in case of unresectable or metastatic GIST, even if some trials are studying its effects after curative resection. GIST of the mesocolon are rare, and as in the other locations, require extensive surgery. The Autohrs report a case of giant malignant GIST arising from transverse mesocolon, treated by en-bloc resection of the tumor with a segment of transverse colon and great omentum.     

影响胃肠道间质瘤预后因素的研究进展

胃肠道间质瘤(GIST)是起源于间叶组织的消化系统肿瘤。影响本病预后的因素有很多,随着病理学技术的发展和最新循证医学证据的提出,人们发现了更多影响GIST预后的因素。因此,本研究总结肿瘤部位、肿瘤大小、核分裂数、肿瘤是否破裂、手术方式、核抗原Ki67、琥珀酸脱氢酶复合物(SDH)、原癌基因C－KIT突变、血小板源性生长因子受体A(PDGFRA)突变表达等因素对GIST预后的影响。     

十二指肠间质瘤16例临床分析

目的 探讨十二指肠间质瘤的诊断和治疗.方法 回顾性分析了16例十二指肠间质瘤治疗的临床资料.结果 十二指肠间质瘤发病部位主要集中在降部和水平部;出血是最常见的临床症状,其次为上腹部疼痛不适,内镜和CT为最常用的辅助检查手段,16例中行胰十二指肠切除2例,十二指肠节段切除9例,肿瘤局部切除5例,术后随访6～42个月,1例复发并出现肝脏转移存活22个月,其余均未发现复发.结论 根据十二指肠间质瘤大小和确切位置应采取不同的手术方式,大部分患者可通过十二指肠节段切除或局部切除达到根治目的,对于病理分级呈高度危险患者术后应给予伊马替尼辅助治疗.     

Gastrointestinal stromal tumors: clinical considerations

Gastrointestinal stromal tumors (61ST) are an heterogeneous group of non epithelial tumors of the gastrointestinal tract. They are peculiar to extreme cellular variability and uncertain malignancy. Gist are rare tumors that arise from primitive mesenchymal cells located in all gastrointestinal tract. Till now they are object of discussion about their origin, diagnostic standards, prognostic factors, histopathological classification. They are more frequently in over 40 years old people without difference in two sex, but they can appear in the child too and in the young man suffering from HIV. The authors relate two cases of recent observation, and discuss on the biological behaviour of these rare tumors.     

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??Diagnosis and therapeutic strategies of duodenal stromal tumor SUO Jian, LI Wei, WANG Da-guang. Department of Gastric and Colorectal Surgery, the First Hospital of Jilin University, Changchun 130021, China
Corresponding author: SUO Jian, E-mail: suojian0066@126.com
Abstract Gastrointestinal stromal tumors (GIST) is the most common mesenchymal tumors of the gastrointestinal tract and occur in any part of the gastrointestinal tract. But the incidence of duodenal stromal tumors is very low. They usually occur in the middle aged and elderly people and clinical symptoms are unrepresentative. The early diagnosis is difficult and should combine abdominal CT, endoscopy and endoscopic ultrasonography, but depends on histopathology and immunohistochemistry finally. Surgical operation is the best effective treament of duodenal stromal tumors, including pancreaticoduodenectomy, subtotal gastrectomy, segmental duodenectomy and simple tumor resection, etc. The individualized, normative operation and adjuvant therapy of duodenal stromal tumors are according to the size, location, adjacent organs of tumors and their genetic test results.     

Laparoscopic approach of gastric gastrointestinal stromal tumors (GISTs): is it still a courageous choice? Report of two cases

Gastrointestinal stromal tumors (GISTs) are a well-defined clinicopathologic and molecular tumor entity, representing the most common gastrointestinal mesenchymal neoplasm. Differential diagnosis between GIST and other mesenchymal malignancies is crucial, given the successful management using targeted therapy in metastatic GIST. The mainstay of treatment remains surgery, complete tumor resection being the most important independent prognostic factor. Videolaparoscopic approach is still controversial for the high risk of tumor rupture or bleeding. Here we report 2 cases of GIST surgically resected using a videolaparoscopic approach and discuss the efficacy of this technique in selected patients.     

胃肠管间质瘤的综合治疗进展

胃肠管间质瘤为一种胃肠管最常见的原发性间质细胞肿瘤,具有偶发性及无症状性的特点.外科治疗仍是无转移胃肠管间质瘤的首选治疗.但术后复发率较高.针对肿瘤的分子生物学特点,通过联合分子靶向药物的治疗,能够改善该病的预后.     

Extra-gastrointestinal stromal tumor of the pancreas: case report and a review of the literature

Gastrointestinal (GI) stromal tumors are mesenchymal tumors that arise from the GI tract. In rare cases, these tumors are found in intra-abdominal sites unrelated to the GI tract and are immunohistochemically similar to their GI tract counterparts. Primary pancreatic GI stromal tumors are very rare, with only 4 previous cases reported.