gastrointestinal stromal tumor (gist)

胃肠道间质瘤 (GIST)
  • 文章类型: Case Reports
    胃神经鞘瘤是一种极为罕见的肿瘤,起源于胃肠肠神经系统的肌间神经丛。这些神经鞘瘤最常见的是良性的,据报道发生在平均年龄为58岁的女性患者中。它们通常是偶然发现的,但偶尔会出现腹部不适,阻塞性症状,或者消化道出血.经常,最初的临床考虑是胃肠道间质瘤,这是更常见的。通过显微成像和免疫组织化学染色做出明确的诊断。完整的手术切除,通常用腹腔镜检查,是最明确且通常是治愈性的治疗方法,不需要进一步的后续行动。在这里,我们介绍了第一例也是唯一一例80岁以下的胃神经鞘瘤病例,并讨论了当前诊断和治疗方案的最新情况。
    Gastric schwannomas are an exceedingly rare tumor arising from the myenteric plexus of the gastrointestinal enteric nervous system. These schwannomas are most commonly benign and reported to occur in female patients with a mean age of 58 at presentation. They are most often discovered incidentally, but can occasionally present with abdominal discomfort, obstructive symptoms, or GI bleeding. Frequently, the initial clinical consideration is for a gastrointestinal stromal tumor, which is much more common. A definitive diagnosis is made with microscopic imaging and immunohistochemical staining. Complete surgical resection, typically performed laparoscopically, is the most definitive and usually curative treatment, requiring no further follow-up. Herein, we present the first and only case of gastric schwannoma in an octogenarian and discuss an update on current diagnostic and therapeutic options.
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  • 文章类型: Case Reports
    该病例报告介绍了一名42岁女性的临床细节,该女性以前没有医学问题,该女性表现为以黑素性粪便为特征的上消化道出血(UGIB)。初步检查显示轻度贫血,随后的内窥镜检查发现4厘米的粘膜下胃肿块显示近期出血指标。随后的手术病理证实2级高级别胃肠道间质瘤(GIST),复发风险增加。这种情况的意义在于强调在UGIB的鉴别诊断中考虑GIST的必要性。特别是在没有可识别的危险因素的中年人中,例如近期或慢性非甾体抗炎药(NSAID)的使用,消化性溃疡疾病,或报警症状。早期发现和及时手术干预在提高患者预后方面至关重要。虽然完全切除是治疗的基石,建议高危患者使用伊马替尼辅助治疗,以降低复发风险.
    This case report presents the clinical details of a 42-year-old female without previous medical issues who presented with upper gastrointestinal bleeding (UGIB) characterized by melanotic stools. Initial examination revealed mild anemia and subsequent endoscopy identified a 4 cm submucosal gastric mass displaying recent bleeding indicators. Subsequent surgical pathology confirmed a high-grade gastrointestinal stromal tumor (GIST) of grade 2 with a heightened risk of recurrence. The significance of this case lies in underscoring the necessity of considering GIST in the differential diagnosis of UGIB, particularly among middle-aged individuals with no identifiable risk factors such as recent or chronic non-steroidal anti-inflammatory drug (NSAID) use, peptic ulcer disease, or alarm symptoms. Early detection and prompt surgical intervention assume paramount importance in enhancing patient outcomes. While complete resection stands as the cornerstone of treatment, adjuvant imatinib therapy is recommended for high-risk patients to mitigate the risk of recurrence.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)是胃肠道中最常见的间质瘤,最常见于胃部。晚期GIST患者的标准治疗包括手术切除和伊马替尼治疗。已有病例记录了伊马替尼治疗前原发性GIST和伊马替尼治疗后复发性GIST患者的组织形态学改变。然而,在伊马替尼治疗后,没有文献记载的一例患者原发部位GIST复发伴软骨样分化.在这篇文章中,我们报告了一名58岁患者的偶然发现,该患者在胃内复发性GIST手术切除前接受了两种伊马替尼治疗.我们还通过小型文献综述探讨了已报道的具有软骨样分化的GIST的各种病例,以比较组织形态学。免疫表型,以及这些病例的患者人口统计。这篇文章对于报道伊马替尼治疗后GIST的罕见发现具有重要意义,并强调了伊马替尼治疗后GIST可能获得的各种表现,这些表现排除了另一个恶性过程。如软骨肉瘤。
    Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the gastrointestinal tract and is most commonly seen in the stomach. The standard treatment for patients with advanced GISTs include both surgical resection and imatinib therapy. There have been cases that document the alterations of patients\' GIST histomorphology both with primary GIST prior to imatinib therapy and with recurrent GIST after imatinib therapy. However, there has been no documented case of a patient who has recurrent GIST with chondroid differentiation at the primary site after imatinib therapy. In this article, we report an incidental finding of a 58-year-old patient who had two treatments of imatinib therapy prior to surgical resection of her recurrent GIST in her stomach. We also explore through a mini-literature review the various cases of GIST with chondroid differentiation that have been reported to compare the histomorphology, immunophenotype, and patient demographic of these cases. This article is significant for reporting a rare finding of GIST after imatinib therapy and highlights the various presentations that GIST could acquire after imatinib therapy that exclude another malignant process, such as chondrosarcoma.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)是一种罕见的胃肠道肿瘤。本报告详细介绍了一例涉及一名44岁女性的医疗案例,该女性接受了双侧嗜铬细胞瘤切除术,GIST胃切除术,腹腔镜肾上腺切除术和肠切除术。尽管最初对口服伊马替尼反应积极,由于经济限制,治疗被推迟。这种延迟导致了以腹部GIST转移到腹壁为标志的关键事件,随后的破裂导致腹膜积血,紧急手术。经过充分的术后恢复,她在调整药物治疗前成功出院.
    Gastrointestinal stromal tumors (GISTs) represent a rare form of gastrointestinal neoplasm. This report details a medical case involving a 44-year-old woman who underwent bilateral pheochromocytoma resection, GIST gastrectomy, and laparoscopic adrenalectomy with intestinal resection. Despite an initially positive response to oral imatinib, treatment was delayed due to economic constraints. This delay resulted in a critical event marked by abdominal GIST metastasis to the abdominal wall, subsequent rupture leading to hemoperitoneum, and emergency surgery. Following an adequate postsurgical recovery, she was successfully discharged prior to medication adjustments.
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  • 文章类型: Case Reports
    肝内胆管癌(ICC)是胆管癌的一种相对罕见的亚型,近年来,西方国家的ICC发病率不断上升。手术切除是ICC最有效的治疗方法。然而,考虑到大多数患者在晚期被诊断,总体结果非常差,尽管肝切除,但术后ICC复发率仍然很高。我们报告了一例在切除原始肿瘤后出现的胃转移性ICC,在影像学上具有与胃肠道间质瘤(GIST)高度相似的影像学特征。肝脏转移性ICC的报告病例很少。鉴于转移性胆管癌和其他胃肠道肿瘤之间的生存率存在显着差异,包括GISTS,重要的是通过成像特征来描绘差异。我们进一步讨论了肝内ICC的影像学特征,将其与其他胃肿瘤进行比较和对比。
    Intrahepatic cholangiocarcinoma (ICC) is a relatively rare subtype of cholangiocarcinoma, and there has been an increasing incidence of ICC in Western countries in recent years. Surgical resection is the most effective treatment for ICC. However, overall outcomes are extremely poor given that most patients are diagnosed at an advanced stage, and postoperative ICC recurrence is still very high despite hepatic resection. We report a case of metastatic ICC to the stomach presenting after resection of the original tumor, with imaging characteristics highly resembling gastrointestinal stromal tumor (GIST) on imaging. Reported cases of metastatic ICC to the liver are sparse. Given that there is a significant difference in the survival rate between metastatic cholangiocarcinoma and other tumors arising from the gastrointestinal tract, including GISTs, it is important to delineate the differences via imaging features. We further discuss the imaging characteristics of intrahepatic ICC, comparing and contrasting it to other gastric tumors.
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  • 文章类型: Case Reports
    Background: This case report presents two clinical cases of metastatic refractory gastrointestinal stromal tumor (GIST) with treatment history of 6-14 years. The follow-up treatment of both cases comprised ripretinib dose escalation and its combination with other tyrosine kinase inhibitors (TKIs). To the best of our knowledge, this is the first report that explored ripretinib combination therapy in the late-line treatment of GISTs. Case description: Case-1 represents a 57-year-old female patient who underwent surgical resection for retroperitoneal GIST in 2008. After tumor recurrence in 2009, imatinib was started with complete response for 8 years. Imatinib was followed by sunitinib and regorafenib treatment. In March 2021, due to progressive disease (PD), the patient started ripretinib (150 mg QD) and achieved partial response (PR). Six months later, the patient showed PD. Subsequently, ripretinib dose was increased (150 mg BID) followed by ripretinib (100 mg QD) and imatinib (200 mg QD) combination. CT performed in February 2022 revealed stable lesions with internal visible necrosis. Combination therapy achieved stable disease (SD) for 7 months. On further follow-up in July 2022, the patient showed PD and died in September 2022. Case-2: represents a 73-year-old female patient diagnosed with unresectable duodenal GIST with liver, lung, and lymph node metastases in 2016. After treatment with imatinib, followed by sunitinib, regorafenib, and imatinib rechallenge, ripretinib (150 mg QD) was administered in May 2021, and SD was achieved. Ripretinib dose was increased (200 mg QD) due to PD in December 2021. The tumor showed heterogeneous manifestations, with overall size increase and regression in right posterior lobe. In February 2022, ripretinib (150 mg) plus sunitinib (25 mg) QD was commenced. On follow-up in April 2022, the patient showed slightly improved symptoms with stable hematologic parameters. Combination therapy achieved SD for 5 months and the patient showed PD in July 2022 and discontinued the treatment later. The patient was in poor general condition and was receiving nutritional therapy until last follow-up in October 2022. Conclusion: This case report provides evidence that combination therapy of ripretinib with other TKIs could be an effective late-line treatment option for refractory GIST patients.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)占所有胃肠道肿瘤的不到1%,但它是消化系统最常见的间质瘤。它常见于胃和小肠,在结肠和食道中很少见。此外,乙状结肠间质瘤是相当罕见的,因为结直肠GIST经常发生在直肠。本研究共观察了21例患者(包括研究病例),其中男性14人(66.6%),女性7人(33.3%)。我们专注于GIST,并对所有案例介绍进行了在线搜索和系统分析。
    Gastrointestinal stromal tumor (GIST) makes up less than 1% of all gastrointestinal tumors, but it is the most common mesenchymal tumor of the digestive system. It is commonly found in the stomach and the small intestine and rarely seen in the colon and the esophagus. Additionally, sigmoid GIST is quite rare since colorectal GIST often occurs in the rectum. A total of 21 patients (including the study case) were looked at for this study, of which 14 (66.6%) were males and seven (33.3%) were females. We focused on GIST and conducted an online search and systematic analysis of all case presentations.
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  • 文章类型: Journal Article
    UNASSIGNED:关于内镜切除术(ER)和腹腔镜切除术(LR)治疗胃肠道间质瘤(GISTs)的疗效和安全性仍存在临床争议。本研究旨在通过比较ER与LR的相对结局来评估ER治疗GIST的安全性和有效性。
    未经授权:PubMed,WebofScience,科克伦图书馆,Embase被搜查了.从2010年1月至2020年1月检索数据,并根据ER和LR的术中和术后结果进行荟萃分析。干预臂由LR治疗,而比较臂由ER治疗。根据纳入标准选择了相关文献,数据被提取,并对纳入文献进行质量评价。采用纽卡斯尔-渥太华量表(NOS)评估纳入研究的质量。使用Cochraneχ2检验和I2统计量评估研究之间的异质性,漏斗图和Egger检验用于检测发表偏倚。
    未经评估:本分析包括13项研究,共有1,261名患者,(ERvs.LR:543vs.718).术后并发症的发生率[比值比(OR),0.400;P=0.001]在ER组明显更低[3.3%;95%置信区间(CI),LR组的0.015~0.055](8.9%;95%CI,0.03~0.17)。荟萃分析显示,ER后的复发率(1.7%;95%CI,0.005至0.033)低于LR后的复发率(2.5%;95%CI,0.012至0.041)。ER的R0切除率(99%;95%CI,0.975至0.999)与LR的R0切除率(100%;95%CI,0.995至1.000)相似。本研究无发表偏倚(P>0.10),敏感性分析表明该研究是稳健的。
    未经评估:就所有结果而言,ER比LR更安全,更有效,除R0切除率外。因此,应考虑ER的治疗选择。然而,应注意ER后的手术切缘状况。
    UNASSIGNED: There are still clinical controversy on the efficacy and safety of endoscopic resection (ER) and laparoscopic resection (LR) in the treatment of gastrointestinal stromal tumors (GISTs). The present study aimed to evaluate the safety and efficacy of ER in the treatment of GISTs by comparing the relative outcomes of ER to LR.
    UNASSIGNED: PubMed, Web of Science, Cochrane Library, and Embase were searched. Data were retrieved from January 2010 to January 2020 and subjected to a meta-analysis based on the intraoperative and postoperative outcomes of ER and LR. The intervention arm was treated by LR while the comparator arm was treated by ER. Relevant literature was selected based on the inclusion criteria, data was extracted, and quality evaluation of the included literature was carried out. The Newcastle-Ottawa Scale (NOS) was applied for assessing the quality of included studies. Heterogeneity between studies was assessed using the Cochrane χ2 test and I2 statistic, and Funnel plots and Egger\'s test were used to detect publication bias.
    UNASSIGNED: The present analysis included 13 studies, comprising a total of 1,261 patients, (ER vs. LR: 543 vs. 718). The incidence rate of postoperative complications [odds ratio (OR), 0.400; P=0.001] was significantly lower in the ER group [3.3%; 95% confidence interval (CI), 0.015 to 0.055] than the LR group (8.9%; 95% CI, 0.03 to 0.17). The meta-analysis revealed that the recurrence rate following ER (1.7%; 95% CI, 0.005 to 0.033) was lower than that following LR (2.5%; 95% CI, 0.012 to 0.041). The R0 resection rate of ER (99%; 95% CI, 0.975 to 0.999) was similar to that of LR (100%; 95% CI, 0.995 to 1.000). No publication bias in this study (P>0.10), and the sensitivity analysis showed that the study was robust.
    UNASSIGNED: ER was safer and more efficient than LR in terms of all the outcomes, except the R0 resection rate. Thus, ER should be considered the treatment of choice. However, attention should be paid to the surgical margin status following ER.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GISTs)是一种罕见的肿瘤,具有很高的恶性转化风险。大多数GIST是无症状的。鉴于GIST对传统的化疗和放疗具有抵抗力,手术切除仍然是治疗的主要手段。在过去的二十年里,酪氨酸激酶抑制剂(TKI)靶向治疗的发现和广泛的肿瘤突变分析改变了GIST的治疗方式.我们介绍了一例患者,该患者的影像学检查结果与有关妇科恶性肿瘤的癌性腹膜炎一致,但后来被发现患有无法切除的GIST,并因TKI局部消退。
    Gastrointestinal stromal tumors (GISTs) are a rare type of tumor with a high risk of malignant transformation. The majority of GISTs are asymptomatic. Surgical resection remains the mainstay of treatment given that GIST is resistant to traditional chemotherapy and radiotherapy. In the last two decades, the discovery of targeted therapy with tyrosine kinase inhibitor therapy (TKI) and widespread mutation analysis of tumors have transformed the treatment of GIST. We present a case of a patient in whom imaging findings were consistent with carcinomatous peritonitis concerning a gynecological malignancy but who was later found to have an unresectable GIST which locally regressed with TKI.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)是罕见的肿瘤,发病率越来越高。GIST是胃肠道最常见的间质瘤,涉及老年人群,进展缓慢。它起源于Cajal的间质细胞。在胃肠道外发展并且与胃肠管状器官的肠壁或浆膜表面没有连接的GIST被称为肠外胃肠道间质瘤(EGIST)。它们具有与GIST相似的形态学和免疫组织学特征。这里,我们描述了一个在44岁的非裔美国男性中发生的极具侵袭性的肠系膜GIST的独特病例.患者出现在医院,抱怨与50磅体重减轻有关的全身性腹痛,食欲下降,还有便秘.他接受了腹部和骨盆的计算机断层扫描(CT),显示沿中央肠系膜有一个大肿块,约15×11cm,并伴有相邻的转移性淋巴结疾病。他接受了腹部肿块的CT引导活检,组织病理学结果为c-kit(CD117)阳性,并在GIST-1(DOG-1)上发现与GIST一致。根据TNM分期,他的肿瘤为T4级,N1级,考虑到淋巴结受累,他处于IV期。他被转诊给肿瘤科医生,并开始接受伊马替尼的新辅助治疗。肠系膜EGIST,虽然罕见,已知与其他EGIST相比,预后较差;因此,必须迅速采取行动积极治疗这些肿瘤。有丝分裂指数和肿瘤大小等因素影响肠系膜GIST的预后。
    Gastrointestinal stromal tumors (GISTs) are rare tumors with increasing incidence. GIST is the most common mesenchymal tumor of the gastrointestinal tract involving the elderly population with a slow progression. It originates from the interstitial cells of Cajal. GISTs that develop outside the gastrointestinal tract and have no connections with the intestinal walls or serosal surfaces of the gastrointestinal tubular organs are referred to as extraintestinal gastrointestinal stromal tumors (EGISTs). They have similar morphological and immunohistological characteristics as GISTs. Here, we describe a unique case of an extremely aggressive mesenteric GIST in a 44-year-old African American male. The patient presented to the hospital with complaints of generalized abdominal pain associated with 50-pound weight loss, decreased appetite, and constipation. He underwent computed tomography (CT) of the abdomen and pelvis which showed a large mass along the central mesentery measuring about 15 × 11 cm with adjacent metastatic nodal disease. He underwent a CT-guided biopsy of his abdominal mass with histopathology findings positive for c-kit (CD117) and discovered on GIST-1 (DOG-1) consistent with GIST. Based on TNM staging, his tumor was graded T4 with N1 given nodal involvement placing him as a stage IV. He was referred to an oncologist and was started on neoadjuvant therapy with imatinib. Mesenteric EGISTs, while rare, are known to have a worse prognosis compared to other EGISTs; hence, prompt action must be taken in aggressively treating these tumors. Factors such as mitotic index and tumor size affect the prognosis of mesenteric GISTs.
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