This editorial discusses Wang et al's article on familial gastrointestinal stromal tumors(GISTs).We read with great interest this article concerning the diagnosis,treatment,and post-treatment management of patient...This editorial discusses Wang et al's article on familial gastrointestinal stromal tumors(GISTs).We read with great interest this article concerning the diagnosis,treatment,and post-treatment management of patients with familial GISTs.The actual incidence of GISTs may be underestimated due to diagnostic limitations and the long-term low-risk behavior of some GISTs.The molecular landscape of GISTs is primarily driven by mutations in the KIT and platelet-derived growth factor receptor alpha(PDGFRA)genes.A subset of GISTs without these mutations known as wild-type GISTs,may harbor other rare mutations,impacting their response to targeted therapies.Clinically,patients with GISTs present with nonspecific symptoms,often leading to delayed diagnosis.Genetic predispositions in familial GISTs provide insights into the genetic architecture and extragastrointestinal manifestations of GISTs.Management has evolved from surgical interventions to molecular-based therapies using tyrosine kinase inhibitors.The management of GISTs,especially in familial cases,requires a multidisciplinary approach.Cases of different gene mutations were reported in the same family,suggesting that incorporating genetic testing into routine clinical practice is crucial for the early identification of high-risk individuals and the implementation of tailored surveillance programs.展开更多
BACKGROUND Endoscopic full-thickness resection(EFTR)is increasingly used for treating gastrointestinal stromal tumors(GISTs)in the stomach.AIM To compare the efficacy,tolerability,and clinical outcomes of EFTR vs surg...BACKGROUND Endoscopic full-thickness resection(EFTR)is increasingly used for treating gastrointestinal stromal tumors(GISTs)in the stomach.AIM To compare the efficacy,tolerability,and clinical outcomes of EFTR vs surgical resection(SR)for gastric GISTs.METHODS We collected clinical data from patients diagnosed with GISTs who underwent either EFTR or SR at our hospital from October 2011 to July 2024.Patients were matched in a 1:1 ratio based on baseline characteristics and tumor clinical-pathological features using propensity score matching.We analyzed perioperative outcomes and follow-up data.The primary outcome measure was progressionfree survival(PFS).RESULTS Out of 912 patients,573 met the inclusion criteria.After matching,each group included 95 patients.The EFTR group demonstrated statistically significant advantages over the SR group in average operative time(P<0.001),length of hospital stay(P<0.001),time to resume liquid diet(P<0.001),incidence of adverse events(P=0.031),and hospitalization costs(P<0.001).The en bloc resection rate was significantly different,with SR group at 100%and EFTR group at 93.7%(P=0.038).The median follow-up was 2451.50 days.Recurrence occurred in 3 patients in the EFTR group and 4 patients in the SR group,with no statistically significant difference(P=1.000).Factors associated with PFS included age,tumor size,high-risk category in the modified National Institutes of Health(NIH)risk score,and resection status.Resection status was identified as an independent prognostic factor for PFS(P=0.0173,hazard ratios=0.0179,95%CI:0.000655-0.491).Notably,there was no statistically significant difference in PFS between the two groups.CONCLUSION This study is a non-inferiority design.The EFTR group significantly outperformed the SR group in terms of operative time,length of hospital stay,time to resume a liquid diet,incidence of adverse events,and hospitalization costs,demonstrating its higher economic efficiency and better tolerability.Additionally,although the en bloc resection rate was lower in the EFTR group compared to the SR group,there were no significant differences in tumor recurrence rates and progression-free survival between the two groups.This study found no statistical difference in the primary endpoint of postoperative recurrence rates between the two groups.However,due to sample size limitations,this result requires further validation in larger-scale studies.The current results should be viewed as exploratory evidence.展开更多
In this editorial we comment on the article published in the recent issue of World Journal of Gastrointestinal Oncology.This study aims to explore the relationship be-tween preoperative inflammation markers and the re...In this editorial we comment on the article published in the recent issue of World Journal of Gastrointestinal Oncology.This study aims to explore the relationship be-tween preoperative inflammation markers and the recurrence of gastrointestinal stromal tumors(GIST)after surgery.It is well known that the best-documented prognostic parameters for GIST are mitotic activity,tumor size and anatomical site.Besides,mutation status represents a prognostic as well as predictive factor.This study provides a new tool for postoperative recurrence risk assessment of GIST patients by establishing a line chart prediction model,which is certificated by previous research that high platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio correlated with increased tumour sizes,more advanced tumour stages and mitotic index.However,as a retrospective study,inevitable bias exists in the results;furthermore,the sample size of this study is relatively small,in-fluencing the universality of the results.Moreover,when assessing risk rating and prognosis of GIST,some novel inflammatory makers could be taken into consi-deration,such as proenkephalin and SLITRK3.Overall,this study can offer an additional model for GIST prognosis and recurrence risk assessment,indepen-dent of the traditional prognostic factors of GIST.展开更多
BACKGROUND For patients with advanced gastrointestinal stromal tumors(GISTs)carrying the ckit exon 11 mutation,imatinib(IM)at a standard dosage of 400 mg per day is the preferred first-line treatment.In cases where tr...BACKGROUND For patients with advanced gastrointestinal stromal tumors(GISTs)carrying the ckit exon 11 mutation,imatinib(IM)at a standard dosage of 400 mg per day is the preferred first-line treatment.In cases where treatment with IM fails,there is an urgent need for a more precise assessment method to determine whether to switch therapies or escalate the IM dosage.This approach will enhance clinical decision-making and optimize patient outcomes.AIM To investigate IM plasma concentration’s role in second-line treatment decisions for c-kit 11-mutated advanced GISTs post-IM failure.METHODS Patients with advanced GIST harboring c-kit 11 mutation who experienced failure with IM 400 mg per day as first-line treatment at our hospital were retrospectively analyzed.Patients were categorized into a low plasma(LP)concentration group(LP group,<1100 ng/mL)and high plasma(HP)concentration group(HP group,≥1100 ng/mL).Each group was further subdivided into Group A(dose-escalation group)and Group B(drug-switch group).Baseline characteristics were compared and Kaplan-Meier curves were used to analyze the survival of patients.RESULTS Seventy-five patients were included in the analysis.For the LP group(n=28),Group A(n=14)had longer overall survival(OS)than Group B(n=14)(P=0.02).No differences were observed between the two subgroups in disease control rate(DCR),objective response rate,and progression-free survival(PFS)(P>0.05).For the HP group(n=47),Group B(n=18)had a higher DCR and longer PFS than Group A(n=29)(P=0.008 and P=0.03,respectively).No difference in OS was observed between the two subgroups(P>0.05).CONCLUSION Increasing IM dosage for c-kit 11-mutated advanced GISTs post-IM failure may prolong OS if plasma concentration is<1100 ng/mL.Switching tyrosine kinase inhibitors may improve DCR and PFS if≥1100 ng/mL.展开更多
BACKGROUND Gastrointestinal stromal tumors(GISTs)are caused by mutations in the KIT and platelet derived growth factor receptor alpha genes in approximately 90%of cases.A minority of wild-type GISTs are associated wit...BACKGROUND Gastrointestinal stromal tumors(GISTs)are caused by mutations in the KIT and platelet derived growth factor receptor alpha genes in approximately 90%of cases.A minority of wild-type GISTs are associated with neurofibromatosis type 1(NF1),an autosomal dominant genetic disease resulting from pathogenic mutations in the NF1 gene,which encodes the neurofibromin protein.NF1 patients often exhibit multi-system involvement,with café-au-lait macules and neurofibromas being characteristic symptoms.GISTs are a rare complication of NF1,with the tumors most frequently occurring in the small intestine(90%of cases),while occurrences in the stomach are rare.CASE SUMMARY A 51-year-old woman presented to the emergency department with complaints of dizziness,fatigue,chest tightness,and dark stools.Initial examination revealed a red blood cell count of 1.99×1012/L and a hemoglobin level of 43 g/L.She underwent blood transfusions and fluid replacement to stabilize her condition.Further investigations identified typical café-au-lait macules on her trunk,limbs,and face,along with neurofibromas.Endoscopy showed coffee-colored fluid in the gastric cavity,a large submucosal elevation with an exudative covering,and ulcer formation on the gastric fundus.Exploratory laparoscopy confirmed the tumor’s origin in the gastric fundus,and resection of the giant GIST was performed.Pathological analysis revealed a necrotic GIST measuring 18 cm×14 cm,classified as high-risk,with approximately 5 mitotic figures per 10 high-power fields and no tumor at the margins.Immunohistochemistry results were CD117(+),delay of germination 1(+),CD34(+),and succinate dehydrogenase complex iron sulfur subunit B intact expression.Genetic testing using next-generation sequencing confirmed an NF1 gene mutation.The patient underwent successful tumor resection and was discharged home with postoperative regorafenib therapy.A follow-up at one year showed no recurrence.CONCLUSION Given the diversity of clinical symptoms associated with NF1 and the complexity of NF1-related GISTs,surgical resection with complete tumor removal remains the preferred treatment option.However,the absence of a standardized treatment protocol for adjuvant therapy presents numerous challenges for clinicians.展开更多
BACKGROUND 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...BACKGROUND 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.CASE SUMMARY 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.CONCLUSION 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.展开更多
BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abd...BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity,the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors.CASE SUMMARY The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days.Upon physical examination,the patient had flat and tough abdomen with mild pressing pain at lower abdomen,no obvious abdominal mass was touchable,and shifting dullness was positive.Positron emission tomography-computed tomography(CT)showed that in his peritoneal cavity,there were multiple nodules of various sizes,seroperitoneum,multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules.Plain CT scanning at epigastrium/hypogastrium/pelvic cavity+enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity,peritoneum and right groin.Tumor marker of carbohydrate antigen 125 was 808 U/mL,diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia,and postoperative pathological examination confirmed EGIST.Imatinib was administered with better therapeutic effect.CONCLUSION Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation,and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.展开更多
BACKGROUND Preoperative knowledge of mutational status of gastrointestinal stromal tumors(GISTs)is essential to guide the individualized precision therapy.AIM To develop a combined model that integrates clinical and c...BACKGROUND Preoperative knowledge of mutational status of gastrointestinal stromal tumors(GISTs)is essential to guide the individualized precision therapy.AIM To develop a combined model that integrates clinical and contrast-enhanced computed tomography(CE-CT)features to predict gastric GISTs with specific genetic mutations,namely KIT exon 11 mutations or KIT exon 11 codons 557-558 deletions.METHODS A total of 231 GIST patients with definitive genetic phenotypes were divided into a training dataset and a validation dataset in a 7:3 ratio.The models were constructed using selected clinical features,conventional CT features,and radiomics features extracted from abdominal CE-CT images.Three models were developed:ModelCT sign,modelCT sign+rad,and model CTsign+rad+clinic.The diagnostic performance of these models was evaluated using receiver operating characteristic(ROC)curve analysis and the Delong test.RESULTS The ROC analyses revealed that in the training cohort,the area under the curve(AUC)values for model_(CT sign),model_(CT sign+rad),and modelCT_(sign+rad+clinic)for predicting KIT exon 11 mutation were 0.743,0.818,and 0.915,respectively.In the validation cohort,the AUC values for the same models were 0.670,0.781,and 0.811,respectively.For predicting KIT exon 11 codons 557-558 deletions,the AUC values in the training cohort were 0.667,0.842,and 0.720 for model_(CT sign),model_(CT sign+rad),and modelCT_(sign+rad+clinic),respectively.In the validation cohort,the AUC values for the same models were 0.610,0.782,and 0.795,respectively.Based on the decision curve analysis,it was determined that the model_(CT sign+rad+clinic)had clinical significance and utility.CONCLUSION Our findings demonstrate that the combined modelCT_(sign+rad+clinic)effectively distinguishes GISTs with KIT exon 11 mutation and KIT exon 11 codons 557-558 deletions.This combined model has the potential to be valuable in assessing the genotype of GISTs.展开更多
BACKGROUND Gastric IgG4-related disease(IgG4-RD)is rarely encountered in clinical practice,and especially more so among pediatric patients.To our knowledge,this is the first report of IgG4-RD presenting as a calcifyin...BACKGROUND Gastric IgG4-related disease(IgG4-RD)is rarely encountered in clinical practice,and especially more so among pediatric patients.To our knowledge,this is the first report of IgG4-RD presenting as a calcifying gastric mass in a child.We describe how this entity was difficult to differentiate from a gastrointestinal stromal tumor(GIST)imaging-based approaches.Therefore,this case highlights the importance of considering IgG4-RD in the differential diagnosis of gastric tumor before performing surgical resection,especially to distinguish it from malignancy to avoid unnecessary surgery.CASE SUMMARY The patient suffered from epigastric pain for several days.Panendoscopy and computed tomography scan revealed a submucosal tumor.Differential diagnoses included GIST,leiomyoma,teratoma,and mucinous adenocarcinoma.However,laparoscopic proximal gastrectomy allowed for the definitive diagnosis of IgG4-related stomach disease.CONCLUSION We emphasize the importance of considering IgG4-RD in the differential diagnosis of gastric submucosal tumors before performing surgical resection.展开更多
BACKGROUND Gastrointestinal stromal tumors(GIST)are prevalent neoplasm originating from the gastrointestinal mesenchyme.Approximately 50%of GIST patients experience tumor recurrence within 5 years.Thus,there is a pres...BACKGROUND Gastrointestinal stromal tumors(GIST)are prevalent neoplasm originating from the gastrointestinal mesenchyme.Approximately 50%of GIST patients experience tumor recurrence within 5 years.Thus,there is a pressing need to accurately evaluate risk stratification preoperatively.AIM To assess the application of a deep learning model(DLM)combined with computed tomography features for predicting risk stratification of GISTs.METHODS Preoperative contrast-enhanced computed tomography(CECT)images of 551 GIST patients were retrospectively analyzed.All image features were independently analyzed by two radiologists.Quantitative parameters were statistically analyzed to identify significant predictors of high-risk malignancy.Patients were randomly assigned to the training(n=386)and validation cohorts(n=165).A DLM and a combined DLM were established for predicting the GIST risk stratification using convolutional neural network and subsequently evaluated in the validation cohort.RESULTS Among the analyzed CECT image features,tumor size,ulceration,and enlarged feeding vessels were identified as significant risk predictors(P<0.05).In DLM,the overall area under the receiver operating characteristic curve(AUROC)was 0.88,with the accuracy(ACC)and AUROCs for each stratification being 87%and 0.96 for low-risk,79%and 0.74 for intermediate-risk,and 84%and 0.90 for high-risk,respectively.The overall ACC and AUROC were 84%and 0.94 in the combined model.The ACC and AUROCs for each risk stratification were 92%and 0.97 for low-risk,87%and 0.83 for intermediate-risk,and 90%and 0.96 for high-risk,respectively.Differences in AUROCs for each risk stratification between the two models were significant(P<0.05).CONCLUSION A combined DLM with satisfactory performance for preoperatively predicting GIST stratifications was developed using routine computed tomography data,demonstrating superiority compared to DLM.展开更多
BACKGROUND 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 reg...BACKGROUND 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.AIM To further investigate the mechanism of IGF2 specific to GISTs.METHODS 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.RESULTS 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,OEIGF2 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 overexpressionmediated imatinib resistance in GISTs.CONCLUSION IGF2 targeting of IGF1R signaling inhibited metastasis and decreased imatinib resistance by driving glycolysis in GISTs.展开更多
BACKGROUND Endoscopic resection(ER)and laparoscopic resection(LR)have been widely used for the treatment of non-metastatic gastric gastrointestinal stromal tumors(gGISTs)(2-5 cm),but there are no selection criteria fo...BACKGROUND Endoscopic resection(ER)and laparoscopic resection(LR)have been widely used for the treatment of non-metastatic gastric gastrointestinal stromal tumors(gGISTs)(2-5 cm),but there are no selection criteria for their application.AIM To provide a reference for the development of standardized treatment strategies for gGISTs.METHODS Clinical baseline characteristics,histopathological results,and short-term and long-term outcomes of patients who treated with ER or LR for gGISTs of 2-5 cm in Taizhou Hospital of Zhejiang Province from January 2014 to August 2022 were retrospectively reviewed.Propensity score matching(PSM)was employed to achieve balance in baseline characteristics of the two groups.RESULTS Among 206 patients,135 were in the ER group and 71 in the LR group.The ER group had significantly smaller tumors[3.5 cm(3.0-4.0 cm)vs 4.2 cm(3.3-5.0 cm),P<0.001]and different tumor locations(P=0.048).After PSM,59 pairs of patients were balanced.After matching,the baseline characteristics of the ER and LR groups did not differ significantly from each other.Compared with LR,ER had faster recovery of diet(P=0.046)and fewer postoperative symptoms(P=0.040).LR achieved a higher complete resection rate(P<0.001)and shorter operation time(P<0.001).No significant differences were observed in postoperative hospital stay(P=0.478),hospital costs(P=0.469),complication rates(P>0.999),pathological features(mitosis,P=0.262;National Institutes of Health risk classification,P=0.145),recurrence rates(P=0.476),or mortality rates(P=0.611).CONCLUSION Both ER and LR are safe and effective treatments for gGISTs.ER has less postoperative pain and faster recovery,while LR has a higher rate of complete resection.展开更多
BACKGROUND Gastric stromal tumors,originating from mesenchymal tissues,are one of the most common tumors of the digestive tract.For stromal tumors originating from the muscularis propria,compared with conventional end...BACKGROUND Gastric stromal tumors,originating from mesenchymal tissues,are one of the most common tumors of the digestive tract.For stromal tumors originating from the muscularis propria,compared with conventional endoscopic submucosal dissection(ESD),endoscopic full-thickness resection(EFTR)can remove deep lesions and digestive tract wall tumors completely.However,this technique has major limitations such as perforation,postoperative bleeding,and post-polypectomy syndrome.Herein,we report a case of postoperative serous surface bleeding which formed an encapsulated hemoperitoneum in a patient with gastric stromal tumor that was treated with exposed EFTR.Feasible treatment options to address this complication are described.CASE SUMMARY A 47-year-old male patient had a hemispherical protrusion found during gastric endoscopic ultrasonography,located at the upper gastric curvature adjacent to the stomach fundus,with a smooth surface mucosa and poor mobility.The lesion was 19.3 mm×16.1 mm in size and originated from the fourth ultrasound layer.Computed tomography(CT)revealed no significant evidence of lymph node enlargement or distant metastasis.Using conventional ESD technology for mucosal pre-resection,exposed EFTR was performed to resect the intact tumor in order to achieve a definitive histopathological diagnosis.Based on its morphology and immunohistochemical expression of CD117 and DOG-1,the lesion was proven to be consistent with a gastric stromal tumor.Six days after exposed EFTR,CT showed a large amount of encapsulated fluid and gas accumulation around the stomach.In addition,gastroscopy suggested intracavitary bleeding and abdominal puncture drainage indicated serosal bleeding.Based on these findings,the patient was diagnosed with serosal bleeding resulting in encapsulated abdominal hemorrhage after exposed EFTR for a gastric stromal tumor.The patient received combined treatments,such as hemostasis under gastroscopy,gastrointestinal decompression,and abdominal drainage.All examinations were normal within six months of follow-up.CONCLUSION This patient developed serous surface bleeding in the gastric cavity following exposed EFTR.Serosal bleeding resulting in an encapsulated hemoperitoneum is rare in clinical practice.The combined treatment may replace certain surgical techniques.展开更多
BACKGROUND Imatinib(IMA)has received approval as the primary treatment for gastrointestinal stromal tumors(GIST).Nonetheless,approximately half of the patients with advanced GIST show disease advancement following IMA...BACKGROUND Imatinib(IMA)has received approval as the primary treatment for gastrointestinal stromal tumors(GIST).Nonetheless,approximately half of the patients with advanced GIST show disease advancement following IMA treatment.Presently,the efficacy of secondary and tertiary medications in addressing various GIST secondary mutations is somewhat restricted.Consequently,there is a significant medical demand for the creation of kinase inhibitors that extensively block secondary drug-resistant mutations in advanced GIST.Ripretinib(RPT)is a new,switch-control tyrosine kinase inhibitors that can suppress different mutations of KIT and PDGFRA via a dual mechanism of action.AIM To investigate the literature on RPT to assess an effective,safe,and successful treatment strategy against advanced GIST.METHODS The present systematic review and meta-analysis was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.PubMed,Embase,Cochrane,Web of Science and ClinicalTrials.gov databases were screened from January 1,2003 to May 1,2024.RESULTS A total of 4 studies were included,with a total of 507 patients enrolled.The objective response rate(ORR)of the RPT-treated advanced GIST was 17%(95%CI:0.11-0.27),while the disease control rate(DCR)was 66%(95%CI:0.59-0.73).The overall occurrence of adverse events with varying degrees was 97%(95%CI:0.93-1),whereas that of grade≥3 adverse reactions was 42%(95%CI:0.28-0.63).The sensitivity analysis revealed that omitting some studies did not yield statistically notable variances in the aggregate data regarding the ORR,DCR,and the occurrence of adverse events of grade 3 or higher.The publication bias was absent because no significant asymmetry was observed in Begg’s funnel plot in all studies.CONCLUSION RPT has favorable efficacy profiles in GIST patients,but the adverse reactions are obvious,and patient management needs to be strengthened to achieve better safety and tolerability.展开更多
BACKGROUND Gastrointestinal stromal tumors(GISTs)vary widely in prognosis,and traditional pathological assessments often lack precision in risk stratification.Advanced imaging techniques,especially magnetic resonance ...BACKGROUND Gastrointestinal stromal tumors(GISTs)vary widely in prognosis,and traditional pathological assessments often lack precision in risk stratification.Advanced imaging techniques,especially magnetic resonance imaging(MRI),offer potential improvements.This study investigates how MRI imagomics can enhance risk assessment and support personalized treatment for GIST patients.AIM To assess the effectiveness of MRI imagomics in improving GIST risk stratification,addressing the limitations of traditional pathological assessments.METHODS Analyzed clinical and MRI data from 132 GIST patients,categorizing them by tumor specifics and dividing into risk groups.Employed dimension reduction for optimal imagomics feature selection from diffusion-weighted imaging(DWI),T1-weighted imaging(T1WI),and contrast enhanced T1WI with fat saturation(CET1WI)fat suppress(fs)sequences.RESULTS Age,lesion diameter,and mitotic figures significantly correlated with GIST risk,with DWI sequence features like sphericity and regional entropy showing high predictive accuracy.The combined T1WI and CE-T1WI fs model had the best predictive efficacy.In the test group,the DWI sequence model demonstrated an area under the curve(AUC)value of 0.960 with a sensitivity of 80.0%and a specificity of 100.0%.On the other hand,the combined performance of the T1WI and CE-T1WI fs models in the test group was the most robust,exhibiting an AUC value of 0.834,a sensitivity of 70.4%,and a specificity of 85.2%.CONCLUSION MRI imagomics,particularly DWI and combined T1WI/CE-T1WI fs models,significantly enhance GIST risk stratification,supporting precise preoperative patient assessment and personalized treatment plans.The clinical implications are profound,enabling more accurate surgical strategy formulation and optimized treatment selection,thereby improving patient outcomes.Future research should focus on multicenter studies to validate these findings,integrate advanced imaging technologies like PET/MRI,and incorporate genetic factors to achieve a more comprehensive risk assessment.展开更多
Gastrointestinal stromal tumors(GISTs)are the most common type of soft tissue sarcoma in the gastrointestinal tract.Most GISTs have been attributed to activated gain-of-function mutations in either KIT or platelet-der...Gastrointestinal stromal tumors(GISTs)are the most common type of soft tissue sarcoma in the gastrointestinal tract.Most GISTs have been attributed to activated gain-of-function mutations in either KIT or platelet-derived growth factor receptorα,making these molecular features essential targets for therapeutic interventions.Although surgery is the standard treatment for localized GISTs,patients often experience relapse and disease progression even after surgery.In recent years,targeted therapy has significantly improved the prognosis of patients with advanced GISTs.Imatinib mesylate,a KIT inhibitor,is the first-line treatment for advanced GISTs and has revolutionized the treatment of this disease.However,drug resistance remains a major issue with imatinib treatment,as a significant majority of patients become resistant to imatinib either after initiation or after 2–3 years of treatment.Consequently,novel tyrosine kinase inhibitors such as sunitinib,regorafenib,ripretinib,and avapritinib have been introduced to address drug resistance.Immunotherapy has emerged as a potential approach for the treatment of advanced GISTs.This review comprehensively summarizes the pathogenesis of GISTs and the development of targeted therapies and immunotherapies,provides an overview of the emergence of drug resistance in advanced GISTs,and discusses the challenges and prospects associated with the treatment of GISTs.展开更多
Gastrointestinal stromal tumors(GIST)are the most common mesenchymalderived 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 t...Gastrointestinal stromal tumors(GIST)are the most common mesenchymalderived 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.展开更多
Gastrointestinal stromal tumors(GISTs)are mesenchymal tumors that arise from the gastrointestinal tract.In rare cases,these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract,such as the...Gastrointestinal stromal tumors(GISTs)are mesenchymal tumors that arise from the gastrointestinal tract.In rare cases,these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract,such as the mesentery,omentum and retroperitoneum.However,pancreatic extra-gastrointestinal stromal tumors are extremely rare,with only 14 previous cases reported.A 61-year-old man with no clinical symptoms had a routine check-up,during which an abdominal mass located in the pancreas tail was detected.Abdominal surgery was performed with resection of the pancreas tail and the spleen,and he was diagnosed with lowrisk GISTs.Another 60-year-old man with no clinical symptoms underwent Computed tomography which revealed a well-demarcated tumor,6 cm in diameter,in the head of the pancreas.He was diagnosed with pancreatic GISTs.Here,we describe two rare cases of pancreatic GISTs and review the cases previously reported in the literature.展开更多
Gastrointestinal stromal tumor(GIST)is a rare but an important clinical entity seen in our clinical practice.It is the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the smal...Gastrointestinal stromal tumor(GIST)is a rare but an important clinical entity seen in our clinical practice.It is the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the small intestine.Although the exact prevalence of GIST is not known,the incidence of GIST has been increasing.GISTs arise from interstitial cells of Cajal.Most of the GISTs occur due to mutation in c-kit gene or platelet derived growth factor receptor alpha gene.15%of GISTs do not have these mutations and they are called wildtype GISTs.Almost all GISTs express KIT receptor tyrosine kinase.Histologically,GISTs look like spindle cell tumors most of the time but they can be epitheloid or mixed type.The median size of GISTs varies from 2.7 cm to 8.9 cm.Clinically,patients with small GISTs remain asymptomatic but as the GIST size increases,patients present with various symptoms depending on the location of the GIST.Most of GISTs are located in the stomach or small bowel.Diagnosis is suspected on imaging and endoscopic studies,and confirmed by tissue acquisition with immunohistochemical staining.The aggressiveness of GISTs depends on the size,mitotic index and location.Surgical resection is the treatment of choice.But various endoscopic modalities of resection are increasingly being tried.Tyrosine kinase inhibitors are extremely useful in the management of large GISTs,unresectable GISTs and metastatic GISTs.Treatment options for metastatic GISTs also include radiotherapy,chemotherapy,hepatic artery embolization,chemoembolization and radiofrequency ablation.展开更多
BACKGROUND Primary extra-gastrointestinal stromal tumors(E-GIST)of the liver are rare.The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect.Oncologic surgery followed by ad...BACKGROUND Primary extra-gastrointestinal stromal tumors(E-GIST)of the liver are rare.The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect.Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care.However,under specific circumstances,a cytoreductive approach may represent a therapeutic option.We describe herein the case of an 84-year-old woman who presented with a tender,protruding epigastric mass.Abdominal computed tomography scan revealed a large,heterogeneous mass located across segments III,IV,V,and VIII of the liver.The initial approach was transarterial embolization of the tumor,which elicited no appreciable response.Considering the large size and central location of the tumor and the advanced age of the patient,non-anatomic complete resection was indicated.Due to substantial intraoperative bleeding and hemodynamic instability,only a near-complete resection could be achieved.Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver.Considering the risk/benefit ratio for therapeutic options,debulking surgery may represent a strategy to control pain and prolong survival.CASE SUMMARY Here,we present a case report of a patient diagnosed with E-GIST primary of the liver,which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.CONCLUSION E-GIST primary of the liver is a rare conditional,the treatment is with systemic therapy and total resection surgery.However,a cytoreductive surgery will be necessary when a complete resection is no possible.展开更多
基金National Natural Science Foundation of China,No.82370569Basic and Applied Basic Research Foundation of Guangdong Province,No.2022A1515012647the Key Program for Science and Technology Projects of Social Development in Zhuhai,No.2220004000249(to Li XF).
文摘This editorial discusses Wang et al's article on familial gastrointestinal stromal tumors(GISTs).We read with great interest this article concerning the diagnosis,treatment,and post-treatment management of patients with familial GISTs.The actual incidence of GISTs may be underestimated due to diagnostic limitations and the long-term low-risk behavior of some GISTs.The molecular landscape of GISTs is primarily driven by mutations in the KIT and platelet-derived growth factor receptor alpha(PDGFRA)genes.A subset of GISTs without these mutations known as wild-type GISTs,may harbor other rare mutations,impacting their response to targeted therapies.Clinically,patients with GISTs present with nonspecific symptoms,often leading to delayed diagnosis.Genetic predispositions in familial GISTs provide insights into the genetic architecture and extragastrointestinal manifestations of GISTs.Management has evolved from surgical interventions to molecular-based therapies using tyrosine kinase inhibitors.The management of GISTs,especially in familial cases,requires a multidisciplinary approach.Cases of different gene mutations were reported in the same family,suggesting that incorporating genetic testing into routine clinical practice is crucial for the early identification of high-risk individuals and the implementation of tailored surveillance programs.
文摘BACKGROUND Endoscopic full-thickness resection(EFTR)is increasingly used for treating gastrointestinal stromal tumors(GISTs)in the stomach.AIM To compare the efficacy,tolerability,and clinical outcomes of EFTR vs surgical resection(SR)for gastric GISTs.METHODS We collected clinical data from patients diagnosed with GISTs who underwent either EFTR or SR at our hospital from October 2011 to July 2024.Patients were matched in a 1:1 ratio based on baseline characteristics and tumor clinical-pathological features using propensity score matching.We analyzed perioperative outcomes and follow-up data.The primary outcome measure was progressionfree survival(PFS).RESULTS Out of 912 patients,573 met the inclusion criteria.After matching,each group included 95 patients.The EFTR group demonstrated statistically significant advantages over the SR group in average operative time(P<0.001),length of hospital stay(P<0.001),time to resume liquid diet(P<0.001),incidence of adverse events(P=0.031),and hospitalization costs(P<0.001).The en bloc resection rate was significantly different,with SR group at 100%and EFTR group at 93.7%(P=0.038).The median follow-up was 2451.50 days.Recurrence occurred in 3 patients in the EFTR group and 4 patients in the SR group,with no statistically significant difference(P=1.000).Factors associated with PFS included age,tumor size,high-risk category in the modified National Institutes of Health(NIH)risk score,and resection status.Resection status was identified as an independent prognostic factor for PFS(P=0.0173,hazard ratios=0.0179,95%CI:0.000655-0.491).Notably,there was no statistically significant difference in PFS between the two groups.CONCLUSION This study is a non-inferiority design.The EFTR group significantly outperformed the SR group in terms of operative time,length of hospital stay,time to resume a liquid diet,incidence of adverse events,and hospitalization costs,demonstrating its higher economic efficiency and better tolerability.Additionally,although the en bloc resection rate was lower in the EFTR group compared to the SR group,there were no significant differences in tumor recurrence rates and progression-free survival between the two groups.This study found no statistical difference in the primary endpoint of postoperative recurrence rates between the two groups.However,due to sample size limitations,this result requires further validation in larger-scale studies.The current results should be viewed as exploratory evidence.
文摘In this editorial we comment on the article published in the recent issue of World Journal of Gastrointestinal Oncology.This study aims to explore the relationship be-tween preoperative inflammation markers and the recurrence of gastrointestinal stromal tumors(GIST)after surgery.It is well known that the best-documented prognostic parameters for GIST are mitotic activity,tumor size and anatomical site.Besides,mutation status represents a prognostic as well as predictive factor.This study provides a new tool for postoperative recurrence risk assessment of GIST patients by establishing a line chart prediction model,which is certificated by previous research that high platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio correlated with increased tumour sizes,more advanced tumour stages and mitotic index.However,as a retrospective study,inevitable bias exists in the results;furthermore,the sample size of this study is relatively small,in-fluencing the universality of the results.Moreover,when assessing risk rating and prognosis of GIST,some novel inflammatory makers could be taken into consi-deration,such as proenkephalin and SLITRK3.Overall,this study can offer an additional model for GIST prognosis and recurrence risk assessment,indepen-dent of the traditional prognostic factors of GIST.
文摘BACKGROUND For patients with advanced gastrointestinal stromal tumors(GISTs)carrying the ckit exon 11 mutation,imatinib(IM)at a standard dosage of 400 mg per day is the preferred first-line treatment.In cases where treatment with IM fails,there is an urgent need for a more precise assessment method to determine whether to switch therapies or escalate the IM dosage.This approach will enhance clinical decision-making and optimize patient outcomes.AIM To investigate IM plasma concentration’s role in second-line treatment decisions for c-kit 11-mutated advanced GISTs post-IM failure.METHODS Patients with advanced GIST harboring c-kit 11 mutation who experienced failure with IM 400 mg per day as first-line treatment at our hospital were retrospectively analyzed.Patients were categorized into a low plasma(LP)concentration group(LP group,<1100 ng/mL)and high plasma(HP)concentration group(HP group,≥1100 ng/mL).Each group was further subdivided into Group A(dose-escalation group)and Group B(drug-switch group).Baseline characteristics were compared and Kaplan-Meier curves were used to analyze the survival of patients.RESULTS Seventy-five patients were included in the analysis.For the LP group(n=28),Group A(n=14)had longer overall survival(OS)than Group B(n=14)(P=0.02).No differences were observed between the two subgroups in disease control rate(DCR),objective response rate,and progression-free survival(PFS)(P>0.05).For the HP group(n=47),Group B(n=18)had a higher DCR and longer PFS than Group A(n=29)(P=0.008 and P=0.03,respectively).No difference in OS was observed between the two subgroups(P>0.05).CONCLUSION Increasing IM dosage for c-kit 11-mutated advanced GISTs post-IM failure may prolong OS if plasma concentration is<1100 ng/mL.Switching tyrosine kinase inhibitors may improve DCR and PFS if≥1100 ng/mL.
文摘BACKGROUND Gastrointestinal stromal tumors(GISTs)are caused by mutations in the KIT and platelet derived growth factor receptor alpha genes in approximately 90%of cases.A minority of wild-type GISTs are associated with neurofibromatosis type 1(NF1),an autosomal dominant genetic disease resulting from pathogenic mutations in the NF1 gene,which encodes the neurofibromin protein.NF1 patients often exhibit multi-system involvement,with café-au-lait macules and neurofibromas being characteristic symptoms.GISTs are a rare complication of NF1,with the tumors most frequently occurring in the small intestine(90%of cases),while occurrences in the stomach are rare.CASE SUMMARY A 51-year-old woman presented to the emergency department with complaints of dizziness,fatigue,chest tightness,and dark stools.Initial examination revealed a red blood cell count of 1.99×1012/L and a hemoglobin level of 43 g/L.She underwent blood transfusions and fluid replacement to stabilize her condition.Further investigations identified typical café-au-lait macules on her trunk,limbs,and face,along with neurofibromas.Endoscopy showed coffee-colored fluid in the gastric cavity,a large submucosal elevation with an exudative covering,and ulcer formation on the gastric fundus.Exploratory laparoscopy confirmed the tumor’s origin in the gastric fundus,and resection of the giant GIST was performed.Pathological analysis revealed a necrotic GIST measuring 18 cm×14 cm,classified as high-risk,with approximately 5 mitotic figures per 10 high-power fields and no tumor at the margins.Immunohistochemistry results were CD117(+),delay of germination 1(+),CD34(+),and succinate dehydrogenase complex iron sulfur subunit B intact expression.Genetic testing using next-generation sequencing confirmed an NF1 gene mutation.The patient underwent successful tumor resection and was discharged home with postoperative regorafenib therapy.A follow-up at one year showed no recurrence.CONCLUSION Given the diversity of clinical symptoms associated with NF1 and the complexity of NF1-related GISTs,surgical resection with complete tumor removal remains the preferred treatment option.However,the absence of a standardized treatment protocol for adjuvant therapy presents numerous challenges for clinicians.
基金National Natural Science Foundation of China,No.82160842Clinical Research Project of Research Fund of Gansu Provincial Hospital,No.23GSSYD-17General Program of the Joint Scientific Research Fund,No.23JRRA1521.
文摘BACKGROUND 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.CASE SUMMARY 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.CONCLUSION 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.
文摘BACKGROUND Extragastrointestinal stromal tumors(EGIST)and gastrointestinal stromal tumors are of similar pathological type and form.Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity,the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors.CASE SUMMARY The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days.Upon physical examination,the patient had flat and tough abdomen with mild pressing pain at lower abdomen,no obvious abdominal mass was touchable,and shifting dullness was positive.Positron emission tomography-computed tomography(CT)showed that in his peritoneal cavity,there were multiple nodules of various sizes,seroperitoneum,multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules.Plain CT scanning at epigastrium/hypogastrium/pelvic cavity+enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity,peritoneum and right groin.Tumor marker of carbohydrate antigen 125 was 808 U/mL,diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia,and postoperative pathological examination confirmed EGIST.Imatinib was administered with better therapeutic effect.CONCLUSION Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation,and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.
基金Supported by the National Natural Science Foundation of China Program Grant,No.82203108China Postdoctoral Science Foundation,No.2022M722275+1 种基金Beijing Bethune Charitable Foundation,No.WCJZL202105Beijing Xisike Clinical Oncology Research Foundation,No.Y-zai2021/zd-0185。
文摘BACKGROUND Preoperative knowledge of mutational status of gastrointestinal stromal tumors(GISTs)is essential to guide the individualized precision therapy.AIM To develop a combined model that integrates clinical and contrast-enhanced computed tomography(CE-CT)features to predict gastric GISTs with specific genetic mutations,namely KIT exon 11 mutations or KIT exon 11 codons 557-558 deletions.METHODS A total of 231 GIST patients with definitive genetic phenotypes were divided into a training dataset and a validation dataset in a 7:3 ratio.The models were constructed using selected clinical features,conventional CT features,and radiomics features extracted from abdominal CE-CT images.Three models were developed:ModelCT sign,modelCT sign+rad,and model CTsign+rad+clinic.The diagnostic performance of these models was evaluated using receiver operating characteristic(ROC)curve analysis and the Delong test.RESULTS The ROC analyses revealed that in the training cohort,the area under the curve(AUC)values for model_(CT sign),model_(CT sign+rad),and modelCT_(sign+rad+clinic)for predicting KIT exon 11 mutation were 0.743,0.818,and 0.915,respectively.In the validation cohort,the AUC values for the same models were 0.670,0.781,and 0.811,respectively.For predicting KIT exon 11 codons 557-558 deletions,the AUC values in the training cohort were 0.667,0.842,and 0.720 for model_(CT sign),model_(CT sign+rad),and modelCT_(sign+rad+clinic),respectively.In the validation cohort,the AUC values for the same models were 0.610,0.782,and 0.795,respectively.Based on the decision curve analysis,it was determined that the model_(CT sign+rad+clinic)had clinical significance and utility.CONCLUSION Our findings demonstrate that the combined modelCT_(sign+rad+clinic)effectively distinguishes GISTs with KIT exon 11 mutation and KIT exon 11 codons 557-558 deletions.This combined model has the potential to be valuable in assessing the genotype of GISTs.
文摘BACKGROUND Gastric IgG4-related disease(IgG4-RD)is rarely encountered in clinical practice,and especially more so among pediatric patients.To our knowledge,this is the first report of IgG4-RD presenting as a calcifying gastric mass in a child.We describe how this entity was difficult to differentiate from a gastrointestinal stromal tumor(GIST)imaging-based approaches.Therefore,this case highlights the importance of considering IgG4-RD in the differential diagnosis of gastric tumor before performing surgical resection,especially to distinguish it from malignancy to avoid unnecessary surgery.CASE SUMMARY The patient suffered from epigastric pain for several days.Panendoscopy and computed tomography scan revealed a submucosal tumor.Differential diagnoses included GIST,leiomyoma,teratoma,and mucinous adenocarcinoma.However,laparoscopic proximal gastrectomy allowed for the definitive diagnosis of IgG4-related stomach disease.CONCLUSION We emphasize the importance of considering IgG4-RD in the differential diagnosis of gastric submucosal tumors before performing surgical resection.
基金Supported by The Chinese National Key Research and Development Project,No.2021YFC2500400 and No.2021YFC2500402Tianjin Key Medical Discipline(Specialty)Construction Project,No.TJYXZDXK-009A.
文摘BACKGROUND Gastrointestinal stromal tumors(GIST)are prevalent neoplasm originating from the gastrointestinal mesenchyme.Approximately 50%of GIST patients experience tumor recurrence within 5 years.Thus,there is a pressing need to accurately evaluate risk stratification preoperatively.AIM To assess the application of a deep learning model(DLM)combined with computed tomography features for predicting risk stratification of GISTs.METHODS Preoperative contrast-enhanced computed tomography(CECT)images of 551 GIST patients were retrospectively analyzed.All image features were independently analyzed by two radiologists.Quantitative parameters were statistically analyzed to identify significant predictors of high-risk malignancy.Patients were randomly assigned to the training(n=386)and validation cohorts(n=165).A DLM and a combined DLM were established for predicting the GIST risk stratification using convolutional neural network and subsequently evaluated in the validation cohort.RESULTS Among the analyzed CECT image features,tumor size,ulceration,and enlarged feeding vessels were identified as significant risk predictors(P<0.05).In DLM,the overall area under the receiver operating characteristic curve(AUROC)was 0.88,with the accuracy(ACC)and AUROCs for each stratification being 87%and 0.96 for low-risk,79%and 0.74 for intermediate-risk,and 84%and 0.90 for high-risk,respectively.The overall ACC and AUROC were 84%and 0.94 in the combined model.The ACC and AUROCs for each risk stratification were 92%and 0.97 for low-risk,87%and 0.83 for intermediate-risk,and 90%and 0.96 for high-risk,respectively.Differences in AUROCs for each risk stratification between the two models were significant(P<0.05).CONCLUSION A combined DLM with satisfactory performance for preoperatively predicting GIST stratifications was developed using routine computed tomography data,demonstrating superiority compared to DLM.
文摘BACKGROUND 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.AIM To further investigate the mechanism of IGF2 specific to GISTs.METHODS 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.RESULTS 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,OEIGF2 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 overexpressionmediated imatinib resistance in GISTs.CONCLUSION IGF2 targeting of IGF1R signaling inhibited metastasis and decreased imatinib resistance by driving glycolysis in GISTs.
基金Supported by The Medical Science and Technology Project of Zhejiang Province,China,No.2024KY1792The Health Science and Technology Program of Zhejiang Province,China,No.22PY101+2 种基金The Program of Taizhou Science and Technology Grant,China,No.22ywb08 and No.22ywb09The Scientific Research Fund Program of Enze Medical Center,China,No.22EZB12 and No.22EZC17The Open Fund of Key Laboratory of Key Laboratory of Minimally Invasive Techniques and Rapid Rehabilitation of Digestive System Tumor of Zhejiang Province,China,No.21SZDSYS15.
文摘BACKGROUND Endoscopic resection(ER)and laparoscopic resection(LR)have been widely used for the treatment of non-metastatic gastric gastrointestinal stromal tumors(gGISTs)(2-5 cm),but there are no selection criteria for their application.AIM To provide a reference for the development of standardized treatment strategies for gGISTs.METHODS Clinical baseline characteristics,histopathological results,and short-term and long-term outcomes of patients who treated with ER or LR for gGISTs of 2-5 cm in Taizhou Hospital of Zhejiang Province from January 2014 to August 2022 were retrospectively reviewed.Propensity score matching(PSM)was employed to achieve balance in baseline characteristics of the two groups.RESULTS Among 206 patients,135 were in the ER group and 71 in the LR group.The ER group had significantly smaller tumors[3.5 cm(3.0-4.0 cm)vs 4.2 cm(3.3-5.0 cm),P<0.001]and different tumor locations(P=0.048).After PSM,59 pairs of patients were balanced.After matching,the baseline characteristics of the ER and LR groups did not differ significantly from each other.Compared with LR,ER had faster recovery of diet(P=0.046)and fewer postoperative symptoms(P=0.040).LR achieved a higher complete resection rate(P<0.001)and shorter operation time(P<0.001).No significant differences were observed in postoperative hospital stay(P=0.478),hospital costs(P=0.469),complication rates(P>0.999),pathological features(mitosis,P=0.262;National Institutes of Health risk classification,P=0.145),recurrence rates(P=0.476),or mortality rates(P=0.611).CONCLUSION Both ER and LR are safe and effective treatments for gGISTs.ER has less postoperative pain and faster recovery,while LR has a higher rate of complete resection.
文摘BACKGROUND Gastric stromal tumors,originating from mesenchymal tissues,are one of the most common tumors of the digestive tract.For stromal tumors originating from the muscularis propria,compared with conventional endoscopic submucosal dissection(ESD),endoscopic full-thickness resection(EFTR)can remove deep lesions and digestive tract wall tumors completely.However,this technique has major limitations such as perforation,postoperative bleeding,and post-polypectomy syndrome.Herein,we report a case of postoperative serous surface bleeding which formed an encapsulated hemoperitoneum in a patient with gastric stromal tumor that was treated with exposed EFTR.Feasible treatment options to address this complication are described.CASE SUMMARY A 47-year-old male patient had a hemispherical protrusion found during gastric endoscopic ultrasonography,located at the upper gastric curvature adjacent to the stomach fundus,with a smooth surface mucosa and poor mobility.The lesion was 19.3 mm×16.1 mm in size and originated from the fourth ultrasound layer.Computed tomography(CT)revealed no significant evidence of lymph node enlargement or distant metastasis.Using conventional ESD technology for mucosal pre-resection,exposed EFTR was performed to resect the intact tumor in order to achieve a definitive histopathological diagnosis.Based on its morphology and immunohistochemical expression of CD117 and DOG-1,the lesion was proven to be consistent with a gastric stromal tumor.Six days after exposed EFTR,CT showed a large amount of encapsulated fluid and gas accumulation around the stomach.In addition,gastroscopy suggested intracavitary bleeding and abdominal puncture drainage indicated serosal bleeding.Based on these findings,the patient was diagnosed with serosal bleeding resulting in encapsulated abdominal hemorrhage after exposed EFTR for a gastric stromal tumor.The patient received combined treatments,such as hemostasis under gastroscopy,gastrointestinal decompression,and abdominal drainage.All examinations were normal within six months of follow-up.CONCLUSION This patient developed serous surface bleeding in the gastric cavity following exposed EFTR.Serosal bleeding resulting in an encapsulated hemoperitoneum is rare in clinical practice.The combined treatment may replace certain surgical techniques.
文摘BACKGROUND Imatinib(IMA)has received approval as the primary treatment for gastrointestinal stromal tumors(GIST).Nonetheless,approximately half of the patients with advanced GIST show disease advancement following IMA treatment.Presently,the efficacy of secondary and tertiary medications in addressing various GIST secondary mutations is somewhat restricted.Consequently,there is a significant medical demand for the creation of kinase inhibitors that extensively block secondary drug-resistant mutations in advanced GIST.Ripretinib(RPT)is a new,switch-control tyrosine kinase inhibitors that can suppress different mutations of KIT and PDGFRA via a dual mechanism of action.AIM To investigate the literature on RPT to assess an effective,safe,and successful treatment strategy against advanced GIST.METHODS The present systematic review and meta-analysis was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.PubMed,Embase,Cochrane,Web of Science and ClinicalTrials.gov databases were screened from January 1,2003 to May 1,2024.RESULTS A total of 4 studies were included,with a total of 507 patients enrolled.The objective response rate(ORR)of the RPT-treated advanced GIST was 17%(95%CI:0.11-0.27),while the disease control rate(DCR)was 66%(95%CI:0.59-0.73).The overall occurrence of adverse events with varying degrees was 97%(95%CI:0.93-1),whereas that of grade≥3 adverse reactions was 42%(95%CI:0.28-0.63).The sensitivity analysis revealed that omitting some studies did not yield statistically notable variances in the aggregate data regarding the ORR,DCR,and the occurrence of adverse events of grade 3 or higher.The publication bias was absent because no significant asymmetry was observed in Begg’s funnel plot in all studies.CONCLUSION RPT has favorable efficacy profiles in GIST patients,but the adverse reactions are obvious,and patient management needs to be strengthened to achieve better safety and tolerability.
文摘BACKGROUND Gastrointestinal stromal tumors(GISTs)vary widely in prognosis,and traditional pathological assessments often lack precision in risk stratification.Advanced imaging techniques,especially magnetic resonance imaging(MRI),offer potential improvements.This study investigates how MRI imagomics can enhance risk assessment and support personalized treatment for GIST patients.AIM To assess the effectiveness of MRI imagomics in improving GIST risk stratification,addressing the limitations of traditional pathological assessments.METHODS Analyzed clinical and MRI data from 132 GIST patients,categorizing them by tumor specifics and dividing into risk groups.Employed dimension reduction for optimal imagomics feature selection from diffusion-weighted imaging(DWI),T1-weighted imaging(T1WI),and contrast enhanced T1WI with fat saturation(CET1WI)fat suppress(fs)sequences.RESULTS Age,lesion diameter,and mitotic figures significantly correlated with GIST risk,with DWI sequence features like sphericity and regional entropy showing high predictive accuracy.The combined T1WI and CE-T1WI fs model had the best predictive efficacy.In the test group,the DWI sequence model demonstrated an area under the curve(AUC)value of 0.960 with a sensitivity of 80.0%and a specificity of 100.0%.On the other hand,the combined performance of the T1WI and CE-T1WI fs models in the test group was the most robust,exhibiting an AUC value of 0.834,a sensitivity of 70.4%,and a specificity of 85.2%.CONCLUSION MRI imagomics,particularly DWI and combined T1WI/CE-T1WI fs models,significantly enhance GIST risk stratification,supporting precise preoperative patient assessment and personalized treatment plans.The clinical implications are profound,enabling more accurate surgical strategy formulation and optimized treatment selection,thereby improving patient outcomes.Future research should focus on multicenter studies to validate these findings,integrate advanced imaging technologies like PET/MRI,and incorporate genetic factors to achieve a more comprehensive risk assessment.
基金supported by Quzhou City Jiang District Life Oasis Public Welfare Service Center,Health and Health Development Promotion Project(Oncology Research Special Project,no:BJHA-CRP-027).
文摘Gastrointestinal stromal tumors(GISTs)are the most common type of soft tissue sarcoma in the gastrointestinal tract.Most GISTs have been attributed to activated gain-of-function mutations in either KIT or platelet-derived growth factor receptorα,making these molecular features essential targets for therapeutic interventions.Although surgery is the standard treatment for localized GISTs,patients often experience relapse and disease progression even after surgery.In recent years,targeted therapy has significantly improved the prognosis of patients with advanced GISTs.Imatinib mesylate,a KIT inhibitor,is the first-line treatment for advanced GISTs and has revolutionized the treatment of this disease.However,drug resistance remains a major issue with imatinib treatment,as a significant majority of patients become resistant to imatinib either after initiation or after 2–3 years of treatment.Consequently,novel tyrosine kinase inhibitors such as sunitinib,regorafenib,ripretinib,and avapritinib have been introduced to address drug resistance.Immunotherapy has emerged as a potential approach for the treatment of advanced GISTs.This review comprehensively summarizes the pathogenesis of GISTs and the development of targeted therapies and immunotherapies,provides an overview of the emergence of drug resistance in advanced GISTs,and discusses the challenges and prospects associated with the treatment of GISTs.
基金Supported by the National Natural Science Foundation of China,No.82160842Clinical Research Project of Research Fund of Gansu Provincial Hospital,No.23GSSYD-17.
文摘Gastrointestinal stromal tumors(GIST)are the most common mesenchymalderived 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.
基金Supported by The Beijing Hope Run Special Fund,LC2012A09Beijing Municipal Science and Technology Commission,Z131107002213164
文摘Gastrointestinal stromal tumors(GISTs)are mesenchymal tumors that arise from the gastrointestinal tract.In rare cases,these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract,such as the mesentery,omentum and retroperitoneum.However,pancreatic extra-gastrointestinal stromal tumors are extremely rare,with only 14 previous cases reported.A 61-year-old man with no clinical symptoms had a routine check-up,during which an abdominal mass located in the pancreas tail was detected.Abdominal surgery was performed with resection of the pancreas tail and the spleen,and he was diagnosed with lowrisk GISTs.Another 60-year-old man with no clinical symptoms underwent Computed tomography which revealed a well-demarcated tumor,6 cm in diameter,in the head of the pancreas.He was diagnosed with pancreatic GISTs.Here,we describe two rare cases of pancreatic GISTs and review the cases previously reported in the literature.
文摘Gastrointestinal stromal tumor(GIST)is a rare but an important clinical entity seen in our clinical practice.It is the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the small intestine.Although the exact prevalence of GIST is not known,the incidence of GIST has been increasing.GISTs arise from interstitial cells of Cajal.Most of the GISTs occur due to mutation in c-kit gene or platelet derived growth factor receptor alpha gene.15%of GISTs do not have these mutations and they are called wildtype GISTs.Almost all GISTs express KIT receptor tyrosine kinase.Histologically,GISTs look like spindle cell tumors most of the time but they can be epitheloid or mixed type.The median size of GISTs varies from 2.7 cm to 8.9 cm.Clinically,patients with small GISTs remain asymptomatic but as the GIST size increases,patients present with various symptoms depending on the location of the GIST.Most of GISTs are located in the stomach or small bowel.Diagnosis is suspected on imaging and endoscopic studies,and confirmed by tissue acquisition with immunohistochemical staining.The aggressiveness of GISTs depends on the size,mitotic index and location.Surgical resection is the treatment of choice.But various endoscopic modalities of resection are increasingly being tried.Tyrosine kinase inhibitors are extremely useful in the management of large GISTs,unresectable GISTs and metastatic GISTs.Treatment options for metastatic GISTs also include radiotherapy,chemotherapy,hepatic artery embolization,chemoembolization and radiofrequency ablation.
基金Supported by Fundo de IncentivoàPesquisa(FIPE)/Hospital de Clínicas de Porto Alegre and Universidade Federal do Rio Grande do Sul.
文摘BACKGROUND Primary extra-gastrointestinal stromal tumors(E-GIST)of the liver are rare.The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect.Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care.However,under specific circumstances,a cytoreductive approach may represent a therapeutic option.We describe herein the case of an 84-year-old woman who presented with a tender,protruding epigastric mass.Abdominal computed tomography scan revealed a large,heterogeneous mass located across segments III,IV,V,and VIII of the liver.The initial approach was transarterial embolization of the tumor,which elicited no appreciable response.Considering the large size and central location of the tumor and the advanced age of the patient,non-anatomic complete resection was indicated.Due to substantial intraoperative bleeding and hemodynamic instability,only a near-complete resection could be achieved.Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver.Considering the risk/benefit ratio for therapeutic options,debulking surgery may represent a strategy to control pain and prolong survival.CASE SUMMARY Here,we present a case report of a patient diagnosed with E-GIST primary of the liver,which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.CONCLUSION E-GIST primary of the liver is a rare conditional,the treatment is with systemic therapy and total resection surgery.However,a cytoreductive surgery will be necessary when a complete resection is no possible.
