2021V1美国国家综合癌症网络胃肠间质瘤诊疗指南更新解读

胃肠间质瘤(gastrointestinal stromal tumor,GIST)是胃肠道最常见的间叶源性肿瘤.近20年来,随着对GIST生物学行为认识的不断深入,靶向药物的广泛应用,以及分子病理学、影像学及外科手术等的进步,基于多学科诊疗模式下的GIST全程化管理显著改善病人预后[1].美国国家综合癌症网络(Nat...     

胃肠间质瘤细胞系的初步建立

胃肠道间质瘤(gastrointestinal stromal tumors,GIST)占消化道恶性肿瘤的2.2%。近年来,GIST发病率有逐渐升高的趋势,本院每年收治GIST病人达50～80例,且GIST的耐药问题越来越严重。在国内GIST细胞系的建立尚未见报道,本研究取GIST病人手术切除的新鲜肿瘤标本行原代培养,     

胃及直肠小胃肠间质瘤的治疗争议与对策

近年来,随着对胃肠间质瘤 （GIST）的研究逐渐深入以及检查手段的不断完善,临床上确诊为GIST的病人逐年增多。大部分GIST病人无明显特异症状,尤其直径<2 cm的小GIST更加缺少特异性临床表现,多为偶然发现。对于小GIST的诊断须影像学和病理学检查相配合,必要时行基因检测。目前关于胃及直肠小GIST的治疗尚存在争议,应根据小GIST病变的位置、大小、是否转移与危险度,以及病人临床表现进行综合评估,采取最佳治疗策略,以保证小GIST治疗的安全性及根治性。     

胃肠间质瘤肝转移的规范化治疗

胃肠间质瘤（GIST）肝转移可发生在各个风险级别和各个时期,并且严重影响病人的生存期。GIST中肝转移发生率约为15.9%,其治疗一直以来是外科领域亟待解决的问题。GIST仍以外科治疗为主,手术完整切除肿瘤和术后服用伊马替尼可改善病人预后,提高存活率。其手术方式的选择更多取决于肿瘤的部位和大小。外科治疗联合酪氨酸激酶抑制剂（TKI）治疗是GIST肝转移病人的有效方法,明显延长GIST肝转移病人的生存时间、提高生存质量。TKI联合外科治疗是GIST肝转移的最优策略。     

复发性胃肠间质瘤再次手术

外科手术目前仍是胃肠间质瘤（GIST）病人获得根治的最佳手段,但术后复发转移是GIST病人治疗失败及死亡的主要原因。对于复发性GIST,外科治疗并不是最佳选择,应联合酪氨酸激酶抑制剂甲磺酸伊马替尼,结合病人的不同情况,个体化制定综合治疗方案,以提高病人总生存期。     

伊马替尼耐药晚期胃肠间质瘤治疗策略

胃肠间质瘤（GIST）是消化道最常见的间叶组织来源肿瘤。目前伊马替尼是转移或不可切除GIST的标准一线治疗药物。近10年随着伊马替尼进入GIST的治疗领域,以往不可治愈GIST病人的预后得到明显改善。然而,多数伊马替尼初始治疗有效的晚期GIST,会在2～3年内发展成伊马替尼继发耐药,原因可能与肿瘤中存在继发c-kit基因突变的多细胞克隆有关。舒尼替尼和新近的瑞格非尼均已获美国食品药品监督管理局（FDA）批准分别用于伊马替尼治疗失败的GIST二线和三线治疗,从而拓展了伊马替尼耐药GIST的治疗选择。对于酪氨酸激酶抑制剂治疗基础上出现局部进展的转移GIST,减瘤手术可使病人获益。对于伴发急性肠梗阻、穿孔或出血的晚期GIST,手术可减轻病人症状。     

复发转移性胃肠间质瘤外科治疗临床价值

对于原发的且病灶局限的胃肠间质瘤（GIST）,手术治疗依然是首选的治疗方案。自伊马替尼被用于治疗GIST以来,对于广泛或巨大原发灶,以及复发转移的GIST病人,如何选择手术治疗或靶向药物治疗以及如何把握两者联合治疗,至今仍有争论,尚须进一步探讨。由于伊马替尼对病人的凝血功能可能有影响,对于服药后的GIST病人进行手术治疗时,术中出血量可能较大,所以术前应先停药且纠正凝血功能。     

十二指肠胃肠道间质瘤的外科治疗及预后分析

目的:探讨十二指肠胃肠道间质瘤(gastrointesinal stromal tumor,GIST)的诊断、外科治疗和预后。方法:回顾性分析2006年3月至2012年10月收治11例十二指肠GIST病人的临床资料,并与同期治疗胃和小肠GIST病人的临床特征及预后进行比较。结果:11例病人CT的检出率为100%,内镜病理检出率为25%。均行手术切除治疗。2例行胰十二指肠切除术,7例行肿瘤局部切除术,2例行十二指肠节段切除术;围手术期无病人死亡,2例分别于术后10个月和2年出现复发、转移。十二指肠GIST病人的1年和2年无复发生存率为91%(10/11)和82%(9/11),预后略差于胃和小肠GIST。结论:十二指肠GIST的术前诊断应综合应用内镜和影像学技术,以提高其确诊率。治疗首选手术切除,根据肿瘤位置和大小合理选择手术方法。低危病人手术后基本可以治愈,中、高危病人术后应配合伊马替尼辅助治疗。该病的预后略逊于胃和小肠GIST。     

NCCN《软组织肉瘤临床实践指南（2018年第1版）》胃肠间质瘤内容更新介绍与解读

胃肠间质瘤（GIST）的诊断和治疗已日趋标准化和规范化。近10余年来,随着分子水平发病机制的揭示和小分子酪氨酸激酶受体抑制剂的成功应用,GIST治疗已经成为精准医疗时代最经典的实体肿瘤靶向治疗成功范例之一。2017年10月,美国国家综合癌症网络（NCCN）发布了最新版的《软组织肉瘤临床实践指南（2018年第1版）》,与既往版本相比,新版指南中GIST部分内容更新较多,涵盖了从诊断到治疗以及随访的GIST病人全程管理。     

胃肠间质瘤病理诊断和分子检测

胃肠间质瘤（GIST）的病理诊断和分子检测对临床治疗和预后判断非常重要。从事GIST诊治的临床医生和病理医生均应熟悉GIST的临床病理学特点和分子检测相关内容,并了解一些容易被误诊为GIST的肿瘤类型,避免误诊和误治,使GIST病人从最大程度上获益。     

胃肠道间质瘤46例临床诊治分析

目的探讨胃肠道间质瘤(G IST)的临床特点。方法对1998~2004年收治的46例G IST病人的临床表现、手术处理及病理结果进行回顾性分析。结果46例均行手术治疗。病理诊断良性13例(28.3%),潜在恶性10例(21.7%),恶性23例(50%)。随访时间6个月至6年,7例出现远处转移或局部复发,10例死亡(其中9例为恶性G IST)。结论G IST缺乏特征性临床表现,术前确诊率较低,其确诊依赖病理结果。目前G IST的治疗仍以手术切除为主,对复发或远处转移者积极再次手术可延长生存期。     

胃肠道间质瘤的诊治现状与进展

目的总结胃肠道间质瘤（GIST）的发病机制及流行病学特点,探讨其诊断及治疗并分析其预后。方法复习与GIST的发病机制、流行病学、诊断、治疗及预后方面有关的文献并对其进行综述。结果 GIST为非上皮源性肿瘤,起源于Cajal间叶细胞,是消化道最常见的间叶性肿瘤,约占消化道肿瘤的1%～3%,中位发病年龄为40～60岁,胃为最好发部位。目前认为,GIST的发病机制与原癌基因c-kit或血小板源性生长因子受体α（PDGFRα）基因突变有密切关系,但PDGFRα和c-kit基因突变不会同时出现在同一患者中。GIST的临床表现缺乏特异性,临床诊断主要依靠内镜及影像学技术,最终确诊依靠病理学检查。目前对GIST的治疗以手术与分子靶向药物治疗为主,其预后与肿瘤危险度分级及治疗干预密切相关。结论 GIST是具有恶性潜能的肿瘤,其危险度分级是指导临床治疗及预后评估的重要指标,GIST的预防、诊断、治疗及复发的预防有待进一步研究。     

以便血为首发症状的胃肠道间质瘤20例诊治分析

目的探讨以便血为首发症状的胃肠道间质瘤诊治经验。方法对有完整资料、经术后病理和免疫组织化学证实的20例以便血为首发症状的胃肠道间质瘤临床资料进行回顾性分析。结果本组病人主要临床表现为黑便、腹痛、头晕、贫血。20例中首诊发现确切病灶11例,余9例为多次就诊发现。20例均接受手术治疗,术中发现伴腹腔播散1例。随访8～67个月,1例死亡,复发、转移5例,其余14例均健在,间质瘤无复发。结论对于以便血为首发症状的胃肠道间质瘤,应有序、及时、联合应用各种检查手段,以提高其首诊阳性率和早诊率。     

胃肠道间质瘤36例临床分析

目的总结胃肠道间质瘤(GIST)的临床特点、诊断与治疗方法。方法回顾性分析36例胃肠道间质瘤患者的临床和病理资料。结果36例GIST患者均行手术治疗,其中良性20例,交界性10例,恶性6例。30例患者获随访2~3年,其中2例死于肿瘤复发转移,1例术后28个月复发再行手术治疗。结论GIST术前诊断较困难,确诊依赖于病理学检查。手术是主要治疗方法,分子靶向治疗具有辅助意义。     

多学科合作模式在胃肠间质瘤诊治中的价值与实施

肿瘤的治疗原则已经由单一的外科治疗演变为多学科共同参与的综合治疗。多学科团队协作（MDT）的诊治模式已经在包括乳腺癌、结直肠癌、胃癌等疾病中得到了应用并取得了成功。胃肠间质瘤（GIST）是胃肠道最常见的间叶源性肿瘤,随着分子靶向治疗时代的到来．GIST的诊断治疗也越来越专业化和个体化．MDT模式在GIST的诊断和治疗上尤其显得更加重要和必要．     

胃肠间质瘤的相关研究进展

胃肠道间质瘤（GIST）是最常见的胃肠道间叶源性肿瘤。外科手术仍然是GIST的唯一治愈方法,且对局限性可切除的复发转移的GIST患者,手术仍能使其获益。基因检测不仅对确诊GIST非常重要,且对靶向药物治疗反应及预后的判断具有重要价值。笔者就以上相关进展作一综述。     

胰腺间质瘤的诊治及预后

目的 探讨原发于胰腺的胃肠道外间质瘤的临床特点、诊断治疗和预后.方法 报道本院诊治的1例胰腺恶性间质瘤,并通过Pubmed和国内主要数据库检索,分析该病的临床资料,对其术后复发转移的危险因素进行预测.结果 1980年1月至2010年7月间,国内外共报道胰腺间质瘤16例,其中男7例,女9例,中位年龄56.5(31～72)岁.临床表现无特异性,主要为上腹疼痛不适.术前诊断依赖于影像检查,肿瘤多为囊实性,边界清晰,CT增强后肿瘤周边或实质内出现不规则强化,胰胆管扩张少见.内镜超声引导下细针穿刺吸取细胞学检查(EUS-FNA)有助于术前确诊.15例手术患者中,14例完整切除肿瘤,1例行囊肿空肠内引流.所有患者均经病理和免疫组化证实为胰腺间质瘤.14例患者的随访时间为1～60个月,平均21个月,所有患者均存活,4例出现复发转移.该病多为高度恶性潜质,单因素分析显示核分裂相≥10/50高倍视野(HPF)为术后复发转移的风险因素.结论 胰腺问质瘤是一种少见的恶性程度相对较低的肿瘤,预后较导管腺癌好.正确诊断和根治性切除可改善其预后.术后即使复发转移,也应积极手术切除,高危患者应服用伊马替尼治疗.

Abstract：

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of pancreatic gastrointestinal stromal tumor (GIST). Methods We reported a case and reviewed the medical literature on pancreatic malignant GIST. We searched the Pubmed and main domestic database. The clinical data of the reported cases were studied, and their predictive factors for postoperative recurrence and metastasis were analyzed. Results Between January 1980 and July 2010, 16 cases of pancreatic GIST were reported. There were 7 males and 9 females, with a median age pf 56.5 (31-72)years. The clinical symptoms were nonspecific. The main presentation was upper abdominal pain or discomfort. A preoperative diagnosis was suspected on radiological examination. The tumor mainly appeared as a well-defined solid-cystic mass. Irregular enhancement appeared in the circumferential and solid portion of the tumor on enhanced CT scan sequences. The pancreatic and biliary ducts were rarely dilated. Endoscopic ultrasound guided fine needle aspiration cytology (EUS-FNA) was helpful in preoperative diagnosis. Of the 15 surgical patients, 14 underwent complete resection, while the remaining received cyst-jejunostomy. A correct diagnosis was made on histopathology and immunohisto-chemistry. On a mean follow up of 21 months (range, 1-60) in 14 patients, all patients were alive.Recurrence or metastasis occurred in 4 patients with tumors of high malignant potential. On univariate analysis, the only significant predictor for adverse outcome was mitoses≥10/50 HPF. Conclusions Pancreatic GIST is a rare tumor of relatively low malignant potential. It has a better prognosis than ductal adenocarcinoma. It is important to arrive at a correct diagnosis and treat the tumor with radical resection. Aggressive surgical resection is potentially curative. Imatinib is recommended in the treatment of patients with tumors with high malignant potential.     

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??Updates and interpretations of the NCCN Clinical Practice Guidelines (2018 first version) on gastrointestinal stromal tumor ZHAO Gang, WANG Ming. Department of Gastrointestinal Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China
Corresponding author: ZHAO Gang, E-mail: zhaogang74313@
aliyun.com
Abstract The diagnosis and treatment of gastrointestinal stromal tumor (GIST) is getting more and more standardized. In the last decade, due to the revelation of the molecular mechanism of its tumorigenesis, as well as the effectiveness of tyrosine kinase inhibitors, GIST has become well known as one of the most classical model of targeted therapy on solid tumor in the precision medicine era. The National Comprehensive Cancer Network (NCCN) issued the latest version of clinical practice guideline on soft tissue sarcoma in October 2017. Compared with previous versions, the new version of the guideline has many updates on the field of GIST, covering the whole-course management of GIST patients from diagnosis to treatment and follow-up.     

Gastrointestinal stromal tumor (GIST)--medical rarities?

Although their overall incidence is low, GISTs are distinctive subgroup of gastrointestinal mesenchymal tumors which express CD117 or platelet derived growth factor receptor alpha (PDGFRA). Considered as rare digestive cancers, tumors like schwannomas, neurofibromas, gastrointestinal leiomiomas are now reclassified as GIST based on immunohistochemistry studies. GIST are more frequent in stomach (40-70%), small bowel (20-40%), colon (5-15%), meanwhile locations such as mesentery, omentum, retro peritoneum in less of 5%. 10 GIST patients were surgically managed during 2004-2009. 5 gastric and 5 small bowel GIST. Most with symptomatic disease: palpable tumor, abdominal pain, anemia, fatigue, superior digestive hemorrhage or occlusion. Imagistic diagnosis consisted of: barium swallow, abdominal sonography, CT and PET-CT. Confirmation was made by hystopathological exam and immunohistochemistry. All patients had more or less wide surgical resections. For some patients there was also a specific adjuvant treatment. All patients survived after surgery. The principle of surgery for GIST is RO resection of the tumor. Tumor rupture or R1 resection of the primary tumor has a negative impact on disease free survival. Some patients (great volume tumors, R1 or R2 resection) had adjuvant treatment. Imatinib mesylate and derivates showed a significant improvement of recurrence free survival with one condition: permanent treatment. Surgery remains the mainstay of treatment in patients with localized, resectable GIST. Recurrence rate of 17-21% and 5 years survival rate of 48-70%, even in resectable GIST, impose an adjuvant treatment.     

Surgery and Imatinib in the Management of GIST: Emerging Approaches to Adjuvant and Neoadjuvant Therapy

Gastrointestinal stromal tumor (GIST) is a neoplasm of the gastrointestinal tract, mesentery, or omentum that expresses the protein-tyrosine kinase KIT (CD117) and is the most common mesenchymal tumor arising at these sites. Surgical resection is the first-line intervention for operable GISTs, particularly localized primary tumors, and it was historically the only effective treatment. However, more than half of all GIST patients present with locally advanced, recurrent, or metastatic disease. The 5-year survival rate ranges from 50% to 65% after complete resection of a localized primary GIST and decreases to approximately 35% for patients with advanced disease who undergo complete surgical resection. A total of 40% to 90% of all GIST surgical patients subsequently have postoperative recurrence or metastasis. Imatinib is a potent, specific inhibitor of KIT that has demonstrated significant activity and tolerability in the treatment of malignant unresectable or metastatic GIST, inducing tumor shrinkage of 50% or more or stabilizing disease in most patients. A key strategy for prolonging the survival of patients with GIST is to improve the outcome of surgery. It is possible that the adjuvant and neoadjuvant use of imatinib (e.g., rendering initially inoperable tumors resectable) in the overall management approach to advanced GIST may contribute to surgeons success in attaining this objective.