Gastrointestinal stromal tumors (GISTs) are a type of tumor that originates from gastrointestinal mesenchymal tissue. Although several somatic or germline mutation GIST mice were established, however, there is still a lack of an authentic mice GIST cell lines for further experimental study.

We developed a chemically induced C57BL/6 J GIST model using 3- methylcholanthrene. Tumor characteristics were confirmed through histology and IHC. Primary cells were isolated to establish the mGSTc01 cell line, and molecular profiling was conducted. Additionally, we established GIST model in immunocompetent mice to evaluate their sensitivity to imatinib.

Our study successfully developed a chemically induced murine GIST model, characterized by positive staining of c-kit and DOG-1. The mGSTc01 monoclonal cell line exhibited slender morphology and expressed the c-kit marker, Whole exome sequencing uncovered mutations of Lamb1, MMP9, and c-kit in GIST cells and provided a detailed picture of the entire genome's copy number variations. RNA sequencing indicated genes associated with cell adhesion and focal adhesion were enriched in mGSTc01 cells. The mGSTc01 cells demonstrated obvious malignant behaviors, notably elevated migration, adhesion, and proliferation. In immunocompetent mice, subcutaneous xenografts not only reserved the aggressive phenotype but also displayed a response to imatinib, underscoring the model's applicability for advancing therapeutic research.

We firstly established a mGSTc01 cell line derived from C57BL/6 J mice GIST tumor offers, which closely mimicking human disease characteristics. It is a potent platform for investigating tumor microenvironment of GIST in mice model, and provides a novel way for new therapeutic discoveries in GIST.

Canine soft tissue sarcomas (STSs) are locally aggressive mesenchymal tumors with variable recurrence rates, and often, their therapy is limited to surgical excision. CD117 (KIT) is a tyrosine kinase receptor involved in cell growth and cancer development. c-kit proto-oncogene mutations have been reported to be associated with prognosis and therapy response in human and canine cancers. However, CD117 expression and c-kit mutations have rarely been investigated in canine STSs. This study aims to assess CD117 expression and c-kit mutations in different canine STSs.

Spontaneous STSs were surgically removed, fixed, routinely processed, and stained for histological and anti-CD117 immunohistochemical analyses. Staining intensity and percentage of positivity were scored. Cases with intense CD117 expression in more than 50% of cells were analyzed for the presence of mutations in exons 8, 9, or 11 of the c-kit proto-oncogene.

Overall, 115 canine STSs were collected. Among them, CD117 was expressed in 43 STSs, with diffuse cytoplasmic staining of variable intensity. CD117 was expressed in 16 out of 27 perivascular wall tumors, 12 of 13 sarcomas of fibroblastic origin, 6 of 6 rhabdomyosarcomas, 7 of 46 liposarcomas, and 2 of 3 nerve sheath tumors. Leiomyosarcomas (20 of 20) did not show CD117 expression. Mutations were investigated in 22 cases, all of which returned negative results.

In summary, canine STSs variably expressed CD117, which suggests that tyrosine kinase inhibitors may represent a promising targeted therapy for selected canine STSs histotypes.

Gastrointestinal stromal tumors (GISTs) are sarcomas that arise from the muscular layer of the gastrointestinal tract. The stomach is the most common location, followed by the small intestine. Surgical resection is the cornerstone of treatment; extensive margins and lymphadenectomy are not routinely required. Commonly utilized resection techniques include wedge gastrectomy, excision and closure of the gastrotomy, and anterior gastrotomy access with internal wedge resection. Tyrosine kinase inhibitors can be utilized in the neoadjuvant setting for cases where a reduction in tumor size would optimize resection, and in the adjuvant setting for high-risk tumors. In select cases, metastasectomy may improve prognosis.

Gastrin-releasing peptide receptor (GRPR), overexpressed in various cancers, is a promising target for positron emission tomography (PET). This systematic review investigated the diagnostic value of GRPR-targeted PET imaging in oncology. A systematic search was conducted on major medical databases until May 23, 2024. Keywords were modified to include clinical original studies on GRPR-targeted PET in cancer patients. Out of 1624 searched studies initially, 107 were eligible for the full-text review. Overall, data from 38 studies met inclusion criteria, investigating GRPR-targeting radiotracers in breast cancer, prostate cancer, gastrointestinal stromal tumours (GIST) and gliomas (including optic pathway glioma and glioblastoma multiforme). In breast cancer, GRPR-targeted PET effectively detected primary tumours and metastases, particularly in estrogen receptor (ER)-positive patients, and predicted treatment response. In prostate cancer, high sensitivity (up to 88%) and specificity (up to 90%) for detecting primary tumours were observed, providing added value when combined with magnetic resonance imaging (MRI). In biochemical recurrence, sites of prostate cancer were identified even at PSA levels below 0.5ng/dL. Compared with PSMA PET, GRPR-targeted PET showed comparable or superior detection rates. Considering GIST, GRPR-targeted PET imaging proved to be a valuable diagnostic tool, particularly when [18F] FDG PET results were inconclusive. Regarding gliomas, GRPR-targeted PET achieved a 100% detection rate (MRI reference), aiding localization, preoperative planning, and differentiation between recurrence and malignant transformation. GRPR-targeted PET shows promise in improving cancer diagnostics, particularly in ER-positive breast cancer, prostate cancer, and gliomas, and may enhance clinical decision-making.

Gastrointestinal stromal tumors, the most prevalent mesenchymal tumors (80 %) of the gastrointestinal tract, comprise less than 1 % of all gastrointestinal neoplasms and about 5 % of all sarcomas. Despite their rarity, Gastrointestinal stromal tumors present diverse clinical manifestations, anatomic locations, histological subtypes, and prognostic outcomes.

This scoping review comprehensively explores the epidemiology, clinical characteristics, diagnostic and prognostic modalities, as well as new therapeutic options for Gastrointestinal stromal tumors.

A particular focus is placed on the promising role of bio-nanomaterials as multifunctional agents for drug delivery and 3D tumor microenvironment modeling. Bio-nanomaterials offer promising opportunities for targeted drug delivery, overcoming treatment resistance, and improving therapeutic efficacy.

Despite significant advancements, Gastrointestinal stromal tumors remain a complex clinical entity with ongoing challenges. The integration of nanotechnology into Gastrointestinal stromal tumors management offers the potential to enhance patient outcomes. Future studies should prioritize the development and evaluation of nanomaterial-based therapies in clinical trials to facilitate the translation of laboratory discoveries into real-world clinical applications.

The course of subclinical gastrointestinal stromal tumors (GISTs) is variable. The management of small GISTs is not well-defined.

Records of patients presenting with small GISTs with documented follow-up appointment at our institution between 2016 and 2022 were identified and reviewed. Comparative univariate analysis to compare patient and tumor characteristics and outcomes was performed.

Eighty-six patients were followed for a median of 3.7 years (range 0.1-20 years). The median size at presentation was 1.7 (range 0.1-2.5) cm. A total of 51.2% (n = 44) underwent surgery before or immediately after initial presentation for pain (18.2%), bleeding (15.9%), or patient preference (6.8%). Another 17.4% (n = 15) had delayed surgery for tumor growth (40%), patient preference (2.7%), bleeding (6.7%), or pain (6.7%). The remaining 31.4% (n = 27) of patients never underwent surgery for reasons that included no growth/stability (44.4%), concomitant cancer diagnosis/treatment (29.6%), comorbidities (14.8%), and patient preference (3.7%). Patients who underwent surveillance without intervention compared with those who had delayed surgery were older (71.1 vs. 60.8 years, p < 0.001) with multiple comorbidities or a concurrent cancer diagnosis (70.3% vs. 20%, p = 0.005). There were no differences in survival or rate of distant metastases. Average time to surgery in the delayed group was 2 (range 0.1-10.3) years, and 86% of these patients underwent surgery by 5.5 years after diagnosis.

In older patients with comorbidities or concurrent cancer diagnoses, opting out of surgery does not affect survival. Conversely, younger patients, free from significant comorbidities or other diagnoses, may consider surgery or active surveillance for up to 5 years, with comparable outcomes.

Schwannomas commonly occur in the head and neck region but are rarely seen in the gastrointestinal tract; the stomach and small intestine are the most commonly involved sites. These tumors are usually misdiagnosed as gastrointestinal stromal tumors (GISTs) before histopathological confirmation due to radiological similarity. GI schwannomas show positivity for S100 protein and vimentin but are negative for CD 117 and CD 34, which helps in differentiating the tumor from GISTs. Case 1: a 70-year-old woman was referred to our hospital by complaints of abdominal pain and discomfort. Upper GI endoscopy demonstrated a protruding lesion at the lesser curvature of the gastric body, and fine-needle aspiration biopsy showed chronic inflammation without malignancy. Since the lesion was suspected to be GIST, this patient had surgery, and a gastric schwannoma was resected successfully. Case 2: a 66-year-old female with anemia and abdominal discomfort was found to have a submucosal elevated mass at the greater curvature of the antrum. Fine needle aspiration biopsy was suggestive of a spindle cell tumor resembling GIST. The patient underwent subtotal gastrectomy with Roux-en-Y reconstruction. Histopathology confirmed schwannoma. It is necessary to differentiate gastric schwannomas from other submucosal tumors of the stomach, especially GISTs. Surgical complete resection of schwannomas usually has a good prognosis with a low probability of recurrence. Though rare, gastric schwannomas should be included in the differential diagnosis of submucosal gastric tumors because the correct identification of this tumor type helps in proper management and evasion of unnecessary extensive surgery.

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors that arise from interstitial cells of Cajal. Due to vague presentation, location and confusing imaging studies, they tend to mimic gynaecological tumors. They usually diagnosed intra-operative and histopathology followed by tumor specific receptors such as KIT, CD34, CD 117 and DOG 1 are mainstay of diagnosis of GIST. Prognosis of GISTs depends on mitotic rate, tumor size and organ of origin. Resection of mass with tumor free margins is the target of treatment. L ymphadenectomy is not routine. Chemotherapy with tyrosine kinase inhibitors such as Imatinib, Dasatinib, Sorafenib and follow- up depend upon risk category. In this case series, there were four cases with vague symptoms misdiagnosed as gynaecological tumors are reviewed. Preoperatively tumors assumed to be of gynaecological origin were found to be case of GISTs intra-operatively and confirmed by presence of cajal's cells histopathology and mainly by DOG 1, CD117 and tyrosine kinase inhibitor receptors on immunohistochemistry. All belonged to high risk category of GISTs. Any abdomino-pelvic mass detected on ultrasonography and with unusual presentation presenting at primary health centre the possibility of non-gynecological tumors especially GISTs should be kept in mind and should be referred to higher centres for further investigation and proper management.

Gastrointestinal stromal tumors (GISTs) are difficult to identify the risk level accurately without surgical pathological confirmation. The purpose of our study was to propose a noninvasive prediction method for predicting the malignant potential of GISTs preoperatively by using contrast-enhanced ultrasound (CEUS) with gastric distention.

We reviewed 47 GISTs who underwent CEUS from April 2017 to August 2023 retrospectively, all the lesions were certificated by pathology after surgery. The age of the patient, size of the lesion, shape, necrosis, calcification in the lesion, perfusion parameters including arrival time (AT), peak intensity (PI), time to peak (TTP), and area under the curve (AUC) of the lesion and surrounding normal tissue were analyzed. Logistic regression analyses were performed. Of the 47 GISTs, 26 were high-risk and 21 low-risk tumors respectively.

Compared with low-risk GISTs, high-risk GIST had faster AT (7.7s vs. 11.5s, p < 0.05), higher PI (15.2dB vs. 12.5dB, p < 0.05), and larger size (4.4 cm vs. 2.2 cm, p < 0.001). In multivariate logistic regression, AT, PI, and size were significant features. The corresponding regression equation In (p/(1-p)=-5.9 + 4.5 size + 4.6 PI + 4.0 AT).

The size, AT, and PI of the GISTs on CEUS can be used as parameters for a noninvasive risk level prediction model of GISTs. This model may help identify the different risk levels of GISTs before surgery.

Gastrointestinal stromal tumors (GISTs) arise from the interstitial cells of Cajal, predominantly affecting the stomach and small intestine, with rare occurrences in the duodenum, rectum, and extraintestinal sites. Histologically, GISTs can present as spindle cells, epithelioid cells, or mixed morphologies, with immunohistochemical staining revealing expression of KIT (CD117) and discovered on GIST 1 (DOG1). Approximately 80% of GISTs harbor activating mutations in KIT or platelet derived growth factor receptor α (PDGFRA), which influence their clinical behavior and treatment response. SDH-deficient GISTs, associated with syndromes such as Carney triad and Carney-Stratakis syndrome, represent a distinct subgroup with unique characteristics and management challenges. The standard treatment includes surgery and imatinib for metastatic cases; however, resistance to tyrosine kinase inhibitors remains a significant hurdle, especially in pediatric and wildtype GISTs. This highlights the need for advanced therapeutic strategies and emphasizes the importance of molecular profiling in guiding treatment decisions and improving outcomes for GIST patients.

Patients with gastro-intestinal stromal tumors (GISTs) undergoing tyrosine kinase inhibitor therapy are monitored with regular computed tomography (CT) scans, exposing patients to cumulative radiation. This exploratory study aimed to evaluate circulating tumor DNA (ctDNA) testing to monitor treatment response and compare changes in ctDNA levels with RECIST 1.1 and total tumor volume measurements. Between 2014 and 2021, six patients with KIT proto-oncogene, receptor tyrosine kinase (KIT) exon-11-mutated GIST from whom long-term plasma samples were collected prospectively were included in the study. ctDNA levels of relevant plasma samples were determined using the KIT exon 11 digital droplet PCR drop-off assay. Tumor volume measurements were performed using a semi-automated approach. In total, 94 of 130 clinically relevant ctDNA samples were analyzed. Upon successful treatment response, ctDNA became undetectable in all patients. At progressive disease, ctDNA was detectable in five out of six patients. Higher levels of ctDNA correlated with larger tumor volumes. Undetectable ctDNA at the time of progressive disease on imaging was consistent with lower tumor volumes compared to those with detectable ctDNA. In summary, ctDNA levels seem to correlate with total tumor volume at the time of progressive disease. Our exploratory study shows promise for including ctDNA testing in treatment follow-up.

Gastrointestinal stromal tumors (GISTs) are rare neoplasms affecting the gastrointestinal (GI) tract, primarily in middle-aged and elderly individuals. Originating from interstitial cells of Cajal (ICC), they can develop anywhere in the GI tract, with varying symptoms. Diagnosis involves imaging and tissue acquisition, with surgical excision and tyrosine kinase inhibitors as treatment options. Recurrence rates are high, particularly in larger tumors, which have a significant impact on survival rates. Neoadjuvant therapy may be beneficial in advanced cases. The recent World Health Organization (WHO) classification categorized all GISTs as malignant, emphasizing the importance of prompt diagnosis and management. We are presenting a case of a 24-year-old male who presented with abdominal discomfort and a sensation of pressure while bending forward. The abdominal mass, which was equivalent to the size of a basketball, was disclosed by the physical and radiological examinations. This mass pressurized the surrounding organs and filled the abdominal cavity. During the surgery, we found a well-defined, clearly localized, irregular spherical tumor with two metastases in the greater omentum. The tumor had numerous diverticular expansions of varying diameters. Despite the tumor's substantial size and proximity to adjacent structures and organs, we were able to successfully excise the tumor entirely (R0) and interruptedly suture the duodenum after a partial D4 resection, the tumor's point of origin. The postoperative period was unremarkable. We discharged the patient without any complications and commenced imatinib therapy. During the seven months following the operation, the patient was on adjuvant imatinib therapy and maintained recurrence-free.

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the alimentary tract. The prognosis depends on the primary site, and small intestinal GISTs have a worse prognosis than gastric GISTs. Molecularly targeted drugs to inhibit tyrosine kinase activity of KIT were used for unresectable or recurrent GISTs. However, secondary resistance to the drugs is often acquired, and treatments based on other mechanisms are needed. Previously, we reported that cell adhesion molecule 1 (CADM1) was highly expressed in most of small intestinal GISTs but not in most of gastric GISTs. In the present study, we examined whether the antibody-drug conjugate (ADC) with anti-CADM1 antibody and monomethyl auristatin E (anti-CAD-ADC) shows anti-tumor effect on CADM1-expressing human GIST cells. The ADC adhibited in this study was previously used for CADM1-expressing human mesothelioma cells and showed anti-tumor effect for them in vitro. GIST-T1 cell line of gastric origin which scarcely expresses CADM1 and GIST-T1 cells transfected with CADM1 cDNA (GIST-T1-CAD cells) which highly expresses CADM1 and represents small intestinal GIST were used. In vitro, anti-CAD-ADC showed remarkable cytotoxic activity on GIST-T1-CAD cells, but control ADC did not. Both anti-CAD-ADC and control ADC did not show anti-tumor effect on original GIST-T1 cells. When GIST-T1-CAD cells were subcutaneously injected to the nude mice, intravenous administration of anti-CAD-ADC showed inhibitory effect for tumor enlargement. Tumor of GIST-T1 cells grew even after anti-CAD-ADC injection. When GIST-T1-CAD cells were injected into peritoneal cavity of the SCID mice, intraperitoneal administration of anti-CAD-ADC showed reduction of the peritoneal tumor. On the other hand, peritoneal tumor grew after control ADC administration. Tissue and organ damage due to administration of anti-CAD-ADC was not apparent by macroscopic and histological examinations in mice. These results indicate that anti-CAD-ADC could have apparent anti-tumor effect on CADM1-expressing human GIST cells both in in vitro and in vivo mouse models.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract, and cases of GISTs tend to be of the disseminated type, with a global incidence of 10 to 15 cases/million each year. The rarer familial GISTs, which often represent a population, differ in screening, diagnosis, and treatment. Familial GISTs include primary familial GISTs with predominantly KIT/PDGFRA mutations and wild-type GISTs. However, whether the same genetic family has different phenotypes has not been reported.

We report two cases of rare GISTs in the same family: A male patient with the V561D mutation in exon 12 of the PDGFRA gene, who has been taking the targeted drug imatinib since undergoing surgery, and a female patient diagnosed with wild-type GIST, who has been taking imatinib for 3 years since undergoing surgery. The favorable prognosis of these patients during the 7-year follow-up period validates the accuracy of our treatment strategy, and we have refined the entire process of diagnosis and treatment of familial GISTs in order to better manage this rare familial disease.

Different mutation types of familial GISTs in the same family are very rare, thus it is very important to make the correct diagnosis and treatment strategies according to the results of molecular detection for the management of familial GISTs.

Gastrointestinal stromal tumors (GISTs) are typical gastrointestinal tract neoplasms. Imatinib is the first-line therapy for GIST patients. Drug resistance limits the long-term effectiveness of imatinib. The regulatory effect of insulin-like growth factor 2 (IGF2) has been confirmed in various cancers and is related to resistance to chemotherapy and a worse prognosis.

To further investigate the mechanism of IGF2 specific to GISTs.

IGF2 was screened and analyzed using Gene Expression Omnibus (GEO: GSE225819) data. After IGF2 knockdown or overexpression by transfection, the phenotypes (proliferation, migration, invasion, apoptosis) of GIST cells were characterized by cell counting kit 8, Transwell, and flow cytometry assays. We used western blotting to evaluate pathway-associated and epithelial-mesenchymal transition (EMT)-associated proteins. We injected transfected cells into nude mice to establish a tumor xenograft model and observed the occurrence and metastasis of GIST.

Data from the GEO indicated that IGF2 expression is high in GISTs, associated with liver metastasis, and closely related to drug resistance. GIST cells with high expression of IGF2 had increased proliferation and migration, invasiveness and EMT. Knockdown of IGF2 significantly inhibited those activities. In addition, OE-IGF2 promoted GIST metastasis in vivo in nude mice. IGF2 activated IGF1R signaling in GIST cells, and IGF2/IGF1R-mediated glycolysis was required for GIST with liver metastasis. GIST cells with IGF2 knockdown were sensitive to imatinib treatment when IGF2 overexpression significantly raised imatinib resistance. Moreover, 2-deoxy-D-glucose (a glycolysis inhibitor) treatment reversed IGF2 overexpression-mediated imatinib resistance in GISTs.

IGF2 targeting of IGF1R signaling inhibited metastasis and decreased imatinib resistance by driving glycolysis in GISTs.

Gastrointestinal stromal tumors (GISTs) are the mesenchymal tumors of the gastrointestinal tract. Extra Gastrointestinal stromal tumors (EGISTs) are rare neoplasms that occur outside the gastrointestinal tract [1]. Miettinen et al. [2] described the first EGIST in 1999. EGIST accounts for 10% of all GISTs [3]. The main distinction between GIST and EGIST is the site of origin of the primary tumor, as GIST occurs throughout the GI tract, from the esophagus to the anus, whereas EGIST is a tumor without any connection with the intestinal wall and are reported in the retroperitoneum, mesentery, and omentum [4]. Hu et al. [5] reported a first case report of EGIST of larynx.We present a second case report of Extra-gastrointestinal stromal tumor of larynx a 47-year-old male reported to ENT Department Command Hospital Central Command Lucknow with complaints of Hoarseness. On examination found to have right vocal cord growth for which he underwent excision of right vocal cord growth. Histopathological examination showed typical of a spindle-cell GIST, showing strong immunoreactivity for CD 117, DOG1, Vimentin and CD 99. 6 months follow up post surgery with no recurrence.

A 44-year-old woman was referred to our hospital for the examination and treatment of a presumed gallbladder tumor. Both ultrasound and computed tomography showed an intracystic tumor but failed to point out the discontinuity between the cystic lesion and the gallbladder. Magnetic resonance imaging, however, could clearly depict the presumed intracystic tumor and the discontinuity between the gallbladder and the target lesion. Both contents of the gallbladder and the cystic lesion showed hypo and hyper intense patterns, though both with slightly different intensities, on T1- and T2-weighted images, respectively. Under the preoperative diagnosis of early gallbladder cancer despite these image findings, laparoscopic cholecystectomy was attempted to the patient. Laparoscopic observation, however, revealed that the target lesion was not continuous with the gallbladder and was located in the round ligament of the liver. Intraoperative findings led us to do cholecystectomy and resection of the adjacent cystic tumor. The intracystic tumor was 3 cm in size and had minute solid component inside the cyst wall. Pathological study of the presumed gallbladder cancer showed epithelioid cells and spindle cells growing in sheet like and storiform fashions, respectively. Cystic walls mainly consisted of hypo cellular fibrous components. Immunohistochemical staining of the tumor was positive for CD117 and negative both for desmin and S100, leading to the diagnosis of gastrointestinal stromal tumor. MIB-1 labelling index of the gastrointestinal stromal tumor was 8%. The patient recovered uneventfully and has been well without any recurrences for 3 months.

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal-derived tumors of the GI tract. They can occur throughout the GI tract, and the survival time of some patients can be improved by first-line targeted therapy with imatinib. However, there are some limitations with imatinib treatment. Immunotherapy for GIST has attracted much attention in recent years, and as one of the most abundant cells in the GIST microenvironment, M2 macrophages play an important role in disease progression. They have unique anti-inflammatory and pro-tumorigenic effects and are one target for immunotherapy. This review summarizes the connection between different factors and the programmed death receptor-1/programmed death ligand-1 pathway and M2 macrophages to reactivate or enhance anti-tumor immunity and improve imatinib efficacy, and to provide new ideas for GIST immunotherapy.

Gastrointestinal stromal tumors (GISTs) are a significant subset of mesenchymal tumors primarily found in the gastrointestinal tract, impacting diagnostic and therapeutic approaches. Understanding their epidemiology is crucial for improving patient care and advancing treatment strategies.

Our study at a Saudi tertiary hospital analyzed 50 patients with GIST, focusing on demographics, tumor locations, and risk assessments. We examined predictors of tumor size, including mitosis frequency, and assessed the impact of anatomical location and risk on clinical outcomes using RStudio software (Posit, Boston, MA).

Among 50 patients with GIST, 36 (72.0%) were male with a median age of 60.5 years, and most tumors (33, 66.0%) were in the stomach. Risk assessments categorized tumors as follows: 20 (40.0%) low risk, 12 (24.0%) high risk, 7 (14.0%) moderate risk, 7 (14.0%) very low risk, and 4 (8.0%) no risk. Most tumors were low-grade (41, 82.0%) and nonmetastatic (47, 94.0%), predominantly spindle cell type (37, 74.0%). Tumor size varied significantly across risk categories: high-risk tumors averaged 10.3 cm versus 0.5 cm for no risk and 3.5 cm for very low risk (P < 0.001). Mitosis frequency differed significantly by risk category and tumor grade (P < 0.001). Tumor grade varied notably with risk categories and morphologic types, especially high-grade tumors in high-risk groups (8, 66.7%) and epithelioid tumors (2, 100%). Multivariable analysis identified predictors of tumor size: anatomical location (extra-GI, intra-abdominal; beta = 7.08, P = 0.011) and risk assessment (low risk, beta = 6.91, P = 0.001; moderate risk, beta = 11.2, P < 0.001; high risk, beta = 8.93, P < 0.001). Liver metastasis did not differ significantly across gender, anatomical location, risk assessment, or tumor grade.

In Saudi Arabia, GISTs predominantly affect males and are primarily located in the stomach. Our findings highlight significant variations in tumor size and grade based on risk assessments and anatomical location. Most GISTs were low-grade, nonmetastatic, and spindle cell type, emphasizing the need for enhanced research to improve diagnostics, tailor treatments, and optimize outcomes in the region.

A 7-year-old castrated male Golden Retriever weighing 36.8 kg presented to the Veterinary Teaching Hospital with vomiting, anorexia and depression. After blood tests, radiographic, ultrasound and computed tomography examinations, a 7.85 × 5.90 × 8.75 cm mass was identified in the caecum. To visualise the tumour margin and improve the accuracy of tumour resection, intraoperative short-wave infrared imaging using indocyanine green was performed during surgery. An indocyanine green solution was injected intravenously as a bolus of 5 mg/kg 24 h before surgery. Tumour resection was performed with a 0.5 cm margin from the fluorescent-marked tissues. Histopathological examination revealed a diagnosis of a gastrointestinal stromal tumour (GIST) and the absence of neoplastic cells in the surgical margin, indicating a successful surgery. To our knowledge, this is the first case of a GIST resection in a dog using intraoperative short-wave infrared imaging.

Primary gastrointestinal stromal tumors of the liver are exceedingly rare entities, presenting diagnostic and therapeutic challenges. The authors present a case of a 64-year-old male with a primary gastrointestinal stromal tumor (GIST) of the liver, emphasizing the importance of comprehensive diagnostic evaluation and multidisciplinary management in such uncommon cases.

The patient presented with persistent hypochondriac pain, leading to the discovery of a hepatic mass. Diagnostic work-ups, including imaging studies and biopsy, confirmed the diagnosis of primary GIST in the liver. Following thorough multidisciplinary consultation, the patient underwent right anterior segmentectomy of the liver, performed by our experienced surgeon. Postoperative pathology confirmed the diagnosis of GIST, and the patient was advised to use adjuvant imatinib.

Primary GISTs of the liver pose diagnostic challenges due to their rarity and varied clinical presentations. Imaging modalities, immunohistochemistry, and molecular genotyping are crucial in accurate diagnosis and treatment planning. Surgical resection remains the cornerstone of treatment for localized GISTs, with adjuvant therapy considered based on recurrence risk factors and molecular characteristics.

This case highlights the need for multidisciplinary consultation in managing primary GISTs of the liver. Accurate diagnosis, surgical expertise, and personalized adjuvant therapy are crucial for better patient outcomes. Further research is necessary to enhance our understanding of prognostic factors and treatment strategies for these rare tumors.

Background: The role of robotic surgery for gastrointestinal stromal tumor (GIST) resection remains unclear. This systematic review and meta-analysis aimed to investigate the outcomes of robotic versus laparoscopic surgery in patients requiring surgery for gastric GISTs. Methods: MEDLINE, EMBASE, and the Cochrane databases were searched from inception to September 4, 2023. Two independent reviewers conducted a systematic review of the literature to select all types of analytic studies comparing robotic versus laparoscopic surgery for GISTs and reporting intraoperative, postoperative, and/or pathological outcomes. Results: Overall, 4 retrospective studies were selected, including a total of 264 patients, specifically 111 (42%) in the robotic and 153 (58%) in the laparoscopic group. Robotic surgery was associated with longer operating time (+42.46 min; 95% confidence interval [CI]: 9.34, 75.58; P=0.01; I2: 85%) and reduced use of mechanical staplers (odds ratio [OR]: 0.05; 95%CI: 0.02, 0.11; P<0.00001; I2: 92%;) compared with laparoscopy. Although nonsignificant, conversion to open surgery was less frequently reported for robotic surgery (2.7%) than laparoscopy (5.2%) (OR: 0.59; 95%CI: 0.17, 2.03; P=0.4; I2: 0%). No difference was found for postoperative and oncological outcomes. Conclusions: Robotic surgery for gastric GISTs provides similar intraoperative, postoperative, and pathological outcomes to laparoscopy, despite longer operative time.

Anorectal gastrointestinal stromal tumors are extremely rare, constituting less than 0.1% of rectal tumors. Surgical resection using a transanal wide excision followed by adjuvant therapy with tyrosine kinase inhibitors can be a successful treatment combination to remove the mass and prevent recurrence while preserving the integrity of the anal sphincter.

Gastrointestinal stromal tumors (GISTs) are a rare subset of neoplasms, accounting for about 1%-2% of primary gastrointestinal malignancies. The stomach is the most common site for GISTs, with anorectal GISTs being exceptionally rare, representing only 0.1% of all rectal tumors. The standard approach for managing localized GIST involves complete surgical excision to achieve negative microscopic margins (R0) while preserving the tumor capsule and maintaining anal sphincter function. Surgical resection with transanal wide excision followed by adjuvant therapy using tyrosine kinase inhibitors can successfully remove the mass, prevent recurrence, and preserve the anal sphincter's integrity. Adjuvant therapy with imatinib is the recommended treatment for all localized GISTs assessed to have an intermediate or high risk of relapse. Here, we report a case of a 63-year-old male with a rectal GIST who underwent transanal wide excision followed by adjuvant therapy with tyrosine kinase inhibitors.

Gastrointestinal stromal tumors (GISTs) are neoplasms arising from the bowel wall, most often in the jejunoileum of the small intestine, but rarely from extragastrointestinal locations. GISTs most often occur in patients older than 40 years of age and can present with a multitude of gastrointestinal symptoms. We present a rare case of an extragastrointestinal stromal tumor (EGIST) causing abdominal pain and melena in a 34-year-old Hispanic male. The patient presented with diffuse abdominal pain, melena, and severe anemia. Computed tomography of the abdomen revealed a large mass abutting the small bowel. The patient was taken to surgery where the mass, which appeared to be deriving from the omentum and invading the adjacent small bowel, was completely excised and found to be a spindle cell GIST. Excision margins were determined to be negative, and the patient was started on a tyrosine kinase inhibitor for maintenance therapy. The patient continues to follow up on an outpatient basis for surveillance. This case represents the rare disease entity EGIST presenting outside the typical demographics of the disease in a young patient with no identified previous genetic syndromes. Gross examination of the mass in this case was also atypical given the appearance that the mass was rooted in the omentum and invading the small bowel which would suggest the primary tumor site was extragastrointestinal. This case demonstrates the need to build a differential diagnosis that includes GIST and the ability to successfully treat this disease if it is identified early in the clinical course.

Extragastrointestinal stromal tumors (EGISTs) are rare mesenchymal neoplasms, which develop in the retroperitoneum, mesentery, and omentum, lacking continuity to the stomach or intestines. Authors hereby present a female patient with a large heterogeneous abdominal mass as a case of an omental EGIST. A 46-year-old woman was referred to our hospital due to an insidious enlargement and colicky pain in the right iliac fossa. Abdominal palpation revealed a voluminous, freely mobile, and non-pulsatile mesoabdominal bulge expanding to the hypogastrium. On exploratory midline laparotomy, the tumor was densely fused to the greater omentum, not connected to the stomach, without gross involvement of adjacent structures. The large mass was completely excised after adequate mobilization. Immunohistochemical techniques showed strong and diffuse expression of WT1, actin and DOG-1, as well as multifocal c-KIT marking. Mutational study concluded a double mutation of KIT exon 9 and a mutation of PDGFRA exon 18. The patient was submitted to adjuvant treatment with imatinib mesylate 800 mg/day. Despite an extremely diverse presentation, omental EGISTs often remain clinically silent for a long time having enough space to grow before becoming symptomatic. These tumors have a consistent pattern of metastasis that typically spares lymph nodes unlike epithelial gut neoplasms. Surgery remains the preferred treatment for non-metastatic EGISTs of the greater omentum. It is possible that DOG-1 will supplant KIT as the leading marker in the future. The scarcity of knowledge on omental EGISTs implies a close monitoring of these patients to detect local relapse or distant metastasis.

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors accounting for only a small fraction of all primary malignant tumors of the gastrointestinal tract. Histologically, GISTs are classified as epithelioid, spindle type, or mixed. We present a case of a 66-year-old male incidentally noted to have a pedunculated gastric mass along the lesser curvature of the stomach during a laparoscopic Nissen fundoplication and hiatal hernia repair. A wedge resection was performed and the pathology demonstrated a 3.7 cm GIST of epithelioid type. Four years after the initial surgery, a jejunal mass was identified via CT enterography as part of a workup for ongoing iron deficiency anemia. A laparoscopic small bowel resection was performed, and the pathology revealed a new primary 3.2 cm GIST of the spindle cell subtype. Three years after surgery, surveillance imaging is negative for any recurrence. This appears to be the first report of the occurrence of metachronous primary GISTs of different histologic subtypes, separated by location.

Hollow viscus perforation poses a significant diagnostic and therapeutic dilemma for the majority of clinicians. It is vitally important that in cases of gastrointestinal perforation, the tissue that was perforated is always evaluated, since a malignant tumor can cause this complication as a presentation form. Here, we present the case of a patient whose first manifestation of a malignant gastric tumor was its perforation and the presence of septic shock secondary to this. This case exemplifies the importance of innovative thinking in facilitating a comprehensive diagnostic and therapeutic strategy, leading to the timely identification and management of a malignant tumor by the oncology team; such interventions not only enhance patient outcomes but also mitigate morbidity and mortality rates.

Duodenal GISTs are rare and challenging tumors. Acute life-threatening upper GI bleeding is a possible presentation of duodenal GISTs. Surgery is the standard treatment for localized duodenal GISTs. Imatinib is an effective adjuvant therapy for duodenal GISTs.

GIST is the most common mesenchymal neoplasm of the gastrointestinal tract, accounting for 1%-2% of gastrointestinal tumors. They originate from the interstitial cells of Cajal and are rare in patients younger than 30 years. The stomach is the most common site, followed by the small intestine and colon. GISTs are caused by a gain-of-function mutation in the proto-oncogene receptor tyrosine kinase, with activating mutations in KIT being the most common. Most GISTs are asymptomatic. Even if gastrointestinal bleeding is the most common complication life-threatening hemorrhage is extremely uncommon. We present a case of a 31-year-old male patient presented with massive active hematemesis and melena with hemorrhagic shock. The patient presented with massive hematemesis and melena of 1 h duration. Endoscopy showed pulsating active bleeding from the third part of the duodenum which was difficult to manage via endoscopy. Histopathologic evaluation showed spindle cell type GIST. Intraoperatively, there was a nodular mass with active bleeding on the third part of the duodenum. Duodenectomy with end-to-end anastomosis was done. Discharged with no postoperative complication and was put on imatinib. There are considerable challenges that arise in the diagnosis and treatment of duodenal gastrointestinal stromal tumors (GISTs) when they present with life-threatening upper gastrointestinal hemorrhage. In order to achieve the best possible outcomes for patients, it is crucial to have a comprehensive understanding of the clinical presentation, diagnostic methods, and treatment approaches.

Tumours of the gastrointestinal tract represent nearly a quarter of all newly diagnosed tumours diagnosed in 2019. Various treatment modalities for gastrointestinal cancers exist, some of which may be guided by biomarkers. Biomarkers act as gauges of either normal or pathogenic processes or responses to an exposure or intervention. They come in many forms. This review explores established and potential molecular/immunohistochemical (IHC) predictive and prognostic biomarkers of the gastrointestinal tract.

Advanced and metastatic gastrointestinal stromal tumors (GISTs) presenting with surgical emergencies are rare. Neoadjuvant imatinib being the treatment of choice for non-metastatic advanced disease with a proven role in downstaging the disease may not be feasible in patients presenting with bleeding and obstruction. We present a case series with retrospective analysis of a prospectively maintained database of patients with advanced and metastatic GISTs presenting with surgical emergencies. Clinical characteristics, imaging and endoscopic findings, surgical procedures, histological findings, and outcomes in these patients were studied. Four patients were included in this case series, with three males and one female (age range: 24-60 years). Two patients presented with melena; one with hemodynamic instability despite multiple blood transfusions underwent urgent exploratory laparotomy for bleeding gastric GIST, while the other underwent surgical exploration after careful evaluation given the recurrent, metastatic disease with a stable metabolic response on six months of imatinib. One patient with metastatic jejunal GIST who presented with an umbilical nodule and intestinal obstruction was given a trial of non-operative management for 72 hours, but due to non-resolution of obstruction, segmental jejunal en bloc resection with the dome of the urinary bladder with reconstruction and metastasectomy was needed. The patient with advanced gastric GIST who presented with gastric outlet obstruction was resuscitated, and an attempt of endoscopic naso-jejunal tube placement was tried, which failed, and exploration was needed. The mean length of hospital stay was 7.5 days. Histopathological examination confirmed GIST in all four patients with microscopic negative resection margins. All patients were started on imatinib with dose escalation to 800 mg in the patient with recurrent and metastatic disease; however, the patient with bleeding gastric GIST experienced severe adverse effects of imatinib and discontinued the drug shortly. All four patients are disease-free on follow-ups of 15 months, 48 months for the patient with advanced non-metastatic disease, and six and 24 months for the patients with metastatic disease. In the era of tyrosine kinase inhibitor (TKI) therapy for advanced and metastatic disease, upfront surgery is usually reserved for surgical emergencies only. Surgical resection, the cornerstone for the treatment of resectable GIST, may also be clinically relevant in metastatic settings, although it requires a careful and individualized approach.

Gastrointestinal stromal tumours are rare, but most common mesenchymal tumours originate from the gastrointestinal tract. Though surgery is the primary treatment, advanced tumours require targeted therapy in combination with surgery.

A 62-year-old lady who presented with abdominal distention and pain was found to have a large abdominal mass. A contrast-enhanced computed tomography revealed a large abdominal mass extending from the epigastrium to the pelvis, with a solitary omental deposit. Despite receiving Imatinib for six months, the disease progressed, and she underwent open En block tumour excision with distal gastrectomy and distal pancreatectomy with Roux-en-Y gastrojejunostomy and part of omental resection after multi-disciplinary team discussions. Histological examination confirmed a spindle-type gastrointestinal stromal tumour, which arrived from the stomach's submucosa; immunohistochemistry showed strong cytoplasmic and membranous positivity for CD117.

While rare (0.1-3 % of GI malignancies), GISTs are most common in the stomach (56 %) and small bowel (32 %). Even large tumours can present with vague symptoms without obstructive features. Advanced tumours can be treated with targeted tumour therapy like Imatinib in combination with surgery. Surgical resection, usually laparoscopic, is the gold standard, but open surgery may be needed for large laparoscopically unresectable tumours.

Though large tumours may present with vague symptoms without obstructive features, they tend to be more aggressive and can progress despite imatinib therapy. While laparoscopic surgery is the gold standard, open surgery is preferable for large, laparoscopically unresectable tumours.

A 66-year-old man with background hypertension and type 2 diabetes presented with 1 month history of epigastric pain for investigation. Abdominal CT and MRI identified round homogeneously enhancing mass arising from the lesser curvature of the stomach. On 18 F-FDG PET/CT, the gastric mass demonstrated intense FDG uptake (SUV max , 9.6). The patient subsequently underwent partial gastrectomy, with pathological features and immunohistochemical patterns consistent with gastric schwannoma.

Gastrointestinal stromal tumors (GISTs) are soft tissue sarcomas that originate from the mesenchymal cells of the gastrointestinal tract. Extra-GISTs (EGISTs) are caused by sites outside the gastrointestinal tract. We reported a case of EGIST of the pancreas in a 51-year-old woman. Enhanced CT scan showed a rounded, slightly hypointense focus in the head of the pancreas and the right pars compacta of the descending duodenum. Routine laboratory and endocrine tests were unremarkable. The patient underwent laparoscopic surgery. The diagnosis of EGIST was confirmed through histopathological and immunohistochemical examination. The tumor was found to be CD117+, CD34+, and DOG+ with a high risk of malignancy. No recurrence was observed during the nine-month postoperative follow-up.

Acute gastrointestinal bleeding (GIB) represents a frequently encountered condition that prompts individuals to seek urgent medical attention at the emergency department, often leading to subsequent hospitalization. GIB can range from self-limited bleeding to hemorrhagic shock. Multiple etiologies contribute to the occurrence of GIB. In this report, we present the case of an 84-year-old male with multiple medical comorbidities admitted with hemodynamically stable lower GIB. Colonoscopy demonstrated a submucosal mass without evidence of bleeding. He subsequently underwent an endoscopic ultrasound (EUS) with sonographic findings concerning for a gastrointestinal stromal tumor. However, pathological analysis from both colonoscopy and EUS indicated the presence of blood, but no evidence of malignancy. A follow-up EUS performed two months later showed a complete resolution of the previously observed submucosal mass, suggesting that the initial evaluation was likely a hematoma that has resolved completely.

This study aimed to use conventional ultrasound features, ultrasound radiomics, and machine learning algorithms to establish a predictive model to assess the risk of post-surgical recurrence of gastrointestinal stromal tumors (GISTs).

This retrospective analysis included 230 patients with pathologically diagnosed GISTs. Radiomic features were extracted from manually annotated images. Radiomic features plus conventional ultrasound features were selected using the SelectKbest analysis of variance and stratified tenfold cross-validation recursive elimination methods. Finally, five different machine learning algorithms (logistic regression [LR], support vector machine [SVM], random forest [RF], extreme gradient boosting [XGBoost], and multilayer perceptron [MLP]) were established to predict risk stratification of GISTs. The predictive performance of the established model was mainly evaluated based on the area under the receiver operating characteristic (ROC) curve (AUC) and accuracy, whereas the predictive performance of the optimal machine learning algorithm and a radiologist's subjective assessment were compared using McNemar's test.

Seven radiomics features and one conventional ultrasound feature were selected to construct the machine learning models for GIST risk classification. The mentioned five machine learning models were able to predict the malignant potential of GISTs. LR and SVM outperformed other classifiers on the test set, with LR achieving an accuracy of 0.852 (AUC, 0.881; sensitivity, 0.871; specificity, 0.826) and SVM achieving an accuracy of 0.852 (AUC, 0.879; sensitivity, 0.839; specificity, 0.870), and proved significantly better than the radiologist (accuracy, 0.691; sensitivity, 0.645; specificity, 0.813).

Machine learning-based ultrasound radiomics features are able to noninvasively predict the biological risk of GISTs.

Due to the increasing recognition of gastrointestinal stromal tumor (GIST), novel insights have appeared in both preclinical and clinical research and begun to reshape the field. This study aims to map the research landscape through bibliometric analysis and provide a brief overview for the future of the GIST field.

We searched the Web of Science Core Collection without publication data restrictions for GISTs and performed a bibliometric analysis with CiteSpace and VOSviewer software.

In sum, 5,911 of 13,776 records were included, and these studies were published in 948 journals and written by 24,965 authors from 4,633 institutions in 100 countries. Referring to published reviews and bibliometric analysis, we classified the future trends in four groups. In epidemiological study, precise incidence and clinicopathological features in different regions and races might become potential hotspots. Novel therapy, such as drugs, modified strategies, radioligand therapy, was persistent hotspots in GIST fields, and ctDNA-guided diagnosis, monitoring, and treatment might meet future clinical needs. The debate over serosa surgery vs. mucosa surgery will remain active for a long time in GIST surgery, and function reserve surgery, biology-based surgery will play an important role in future. Moreover, rare GIST type, like NF-1-associated GIST, Carney triads and SDH mutant GIST, need more studies in pathogenesis and genetic mutation to provide appropriate treatment for this orphan GIST patients.

Potential hotspots in future GIST trends might involve epidemiology, agents, resection therapy and rare type GIST, moreover, researchers could pay more attention in these four fields.

Schwannomas of the gastrointestinal tract are rare spindle cell tumors that account for 2-6% of mesenchymal tumors. An elderly male was found to have a left colon mass on CT scan and colonoscopy with pathology of fibrotic tissue. A laparoscopic-assisted left hemi-colectomy with primary anastomosis was performed. Pathology demonstrated spindle cell neoplasm arranged in short fascicles that were strongly and diffusely positive for S100. An elderly female was found to have a submucosal lesion on surveillance colonoscopy in the proximal transverse colon. Biopsy with jumbo forceps revealed spindle cell neoplasm positive for S100. Patient underwent an uncomplicated limited non-oncologic segmental transverse colectomy. We report only the ninth case of left and sixth in the transverse colon described in the literature. As is true for other mesenchymal tumors, mucosal biopsy is usually inconclusive and deep biopsy or submucosal resection is required, making pre-operative surgical decision difficult.

A gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. The tumor originates from Cajal cells, which generated peristaltic motion. Primary gastrointestinal stromal tumor of the liver is rare and should be considered in imaging diagnosis. We report a case of an 82-year-old female patient who represented with upper abdominal pain and poor appetite. Magnetic resonance imaging detected a large left lobe cystic lesion (10 × 15 cm). The tumor was resected. The histopathological and immunohistochemical results were of a primary gastrointestinal stromal tumor in the liver with cystic changes. On imaging, it is difficult to differentiate from other cystic lesions.

Rare gastrointestinal stromal tumors (GISTs) are caused by mutations in the KIT and PDGFRA genes. Avapritinib (BLU-285) is a targeted selective inhibitor for mutated KIT and PDGFRA receptors that can be used to treat these tumors. However, there are subtypes of GISTs that exhibit resistance against BLU-285 and thus require other treatment strategies. This can be addressed by employing a drug delivery system that transports a combination of drugs with distinct cell targets. In this work, we present the synthesis of esterase-responsive polyglycerol-based nanogels (NGs) to overcome drug resistance in rare GISTs. Using inverse nanoprecipitation mediated with inverse electron-demand Diels-Alder cyclizations (iEDDA) between dPG-methyl tetrazine and dPG-norbornene, multi-drug-loaded NGs were formed based on a surfactant-free encapsulation protocol. The obtained NGs displayed great stability in the presence of fetal bovine serum (FBS) and did not trigger hemolysis in red blood cells over a period of 24 h. Exposing the NGs to Candida Antarctica Lipase B (CALB) led to the degradation of the NG network, indicating the capability of targeted drug release. The bioactivity of the loaded NGs was tested in vitro on various cell lines of the GIST-T1 family, which exhibit different drug resistances. Cell internalization with comparable uptake kinetics of the NGs could be confirmed by confocal laser scanning microscopy (CLSM) and flow cytometry for all cell lines. Cell viability and live cell imaging studies revealed that the loaded NGs are capable of intracellular drug release by showing similar IC50 values to those of the free drugs. Furthermore, multi-drug-loaded NGs were capable of overcoming BLU-285 resistance in T1-α-D842V + G680R cells, demonstrating the utility of this carrier system.

Gastrointestinal stromal tumour (GIST) is the most common intra-abdominal sarcoma. Risk classification systems, commonly the modified National Institutes of Health consensus criteria, identify tumour properties relating to patient outcomes. However, owing to limited long-term evidence, most guidelines recommend up to 10-year follow-up for all risk groups except very low-risk GIST.

This retrospective multicentre study included patients who had complete resection of primary, non-metastatic GIST from three Scandinavian sarcoma centres: Gothenburg (2004-2020), Stockholm (2000-2019), and Oslo (2000-2017). Medical records were reviewed for clinical details regarding diagnosis, treatment, and follow-up, and recurrence-free and disease-specific survival evaluated.

The total cohort consisted of 1213 patients with GIST. High-risk patients and those treated with tyrosine kinase inhibitors were excluded. The remaining 649 patients were included in the present analysis: 118 with very low-, 381 with low-, and 150 with intermediate-risk GISTs. Five-year recurrence-free survival rates were 100, 98.5, and 100 per cent for the intermediate-, low-, and very low-risk groups respectively (P = 0.246). Disease-specific survival rates 10 years after surgery were 100, 98.4, and 100 per cent for the intermediate-, low-, and very low-risk groups respectively (P = 0.262).

Patients with completely resected non-high-risk GISTs have an excellent long-term outcome, irrespective of risk group. Follow-up programmes to detect disease recurrences in these patients are probably not indicated.