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1.
Int J Mol Sci ; 25(12)2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38928487

RESUMO

Tissue biopsy remains the standard for diagnosing gastrointestinal stromal tumors (GISTs), although liquid biopsy is emerging as a promising alternative in oncology. In this pilot study, we advocate for droplet digital PCR (ddPCR) to diagnose GIST in tissue samples and explore its potential for early diagnosis via liquid biopsy, focusing on the PDGFRA D842V mutation and SEPT9 hypermethylated gene. We utilized ddPCR to analyze the predominant PDGFRA mutation (D842V) in surgical tissue samples from 15 GIST patients, correlating with pathologists' diagnoses. We expanded our analysis to plasma samples to compare DNA alterations between tumor tissue and plasma, also investigating SEPT9 gene hypermethylation. We successfully detected the PDGFRA D842V mutation in GIST tissues by ddPCR. Despite various protocols to enhance mutation detection in early-stage disease, it remained challenging, likely due to the low concentration of DNA in plasma samples. Additionally, the results of Area Under the Curve (AUC) for the hypermethylated SEPT9 gene, analyzing concentration, ratio, and abundance were 0.74 (95% Confidence Interval (CI): 0.52 to 0.97), 0.77 (95% CI: 0.56 to 0.98), and 0.79 (95% CI: 0.59 to 0.99), respectively. As a rare disease, the early detection of GIST through such biomarkers is particularly crucial, offering significant potential to improve patient outcomes.


Assuntos
Metilação de DNA , Tumores do Estroma Gastrointestinal , Mutação , Reação em Cadeia da Polimerase , Receptor alfa de Fator de Crescimento Derivado de Plaquetas , Septinas , Humanos , Septinas/genética , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Metilação de DNA/genética , Biópsia Líquida/métodos , Projetos Piloto , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética , Feminino , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase/métodos , Idoso , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/patologia , Biomarcadores Tumorais/genética , Adulto
2.
Can Vet J ; 65(6): 553-558, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38827592

RESUMO

Gastrointestinal stromal tumors arising from gastric cardia are uncommon in dogs. A few studies have shown the effectiveness of tyrosine kinase inhibitors in the treatment of canine gastrointestinal stromal tumors, but no standardized protocols are currently available. An 11-year-old spayed female Maltese dog was diagnosed with a gastrointestinal stromal tumor using histopathological and immunohistochemical analyses. An adenosine triphosphate-based tumor chemosensitivity assay revealed that imatinib at lower concentrations had a stronger inhibitory effect than toceranib. Based on the results of the assay, the dog was treated with imatinib after surgery. After 28 mo of therapy, there was no recurrence of the tumor. Key clinical message: Adenosine triphosphate-based tumor chemosensitivity assays may help clinicians to select appropriate postoperative chemotherapeutic drugs for incompletely resected gastrointestinal stromal tumors in dogs.


Gestion réussie à la suite d'une résection incomplète d'une tumeur stromale gastro-intestinale à l'aide de l'imatinib basée sur un test de sensibilité tumorale à base d'adénosine triphosphate chez un chien. Les tumeurs stromales gastro-intestinales résultant du cardia gastrique sont rares chez le chien. Quelques études ont montré l'efficacité des inhibiteurs de la tyrosine kinase dans le traitement des tumeurs stromales gastrointestinales canines, mais aucun protocole standardisé n'est actuellement disponible. Une chienne maltaise stérilisée de 11 ans a reçu un diagnostic de tumeur stromale gastro-intestinale à l'aide d'analyses histopathologiques et immunohistochimiques. Un test de chimiosensibilité tumorale à base d'adénosine triphosphate a révélé que l'imatinib à des concentrations plus faibles avait un effet inhibiteur plus fort que le tocéranib. Sur la base des résultats du test, le chien a été traité avec de l'imatinib après l'opération. Après 28 mois de traitement, il n'y a eu aucune récidive de la tumeur.Message clinique clé :Les tests de chimiosensibilité tumorale à base d'adénosine triphosphate peuvent aider les cliniciens à sélectionner les médicaments chimiothérapeutiques postopératoires appropriés pour les tumeurs stromales gastro-intestinales incomplètement réséquées chez le chien.(Traduit par Dr Serge Messier).


Assuntos
Antineoplásicos , Doenças do Cão , Tumores do Estroma Gastrointestinal , Mesilato de Imatinib , Animais , Tumores do Estroma Gastrointestinal/veterinária , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Cães , Mesilato de Imatinib/uso terapêutico , Doenças do Cão/tratamento farmacológico , Doenças do Cão/cirurgia , Feminino , Antineoplásicos/uso terapêutico , Trifosfato de Adenosina/uso terapêutico , Indóis , Pirróis
3.
J Med Case Rep ; 18(1): 286, 2024 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-38907357

RESUMO

BACKGROUND: Due to rarity of duodenal GISTs, clinicians have few information about its clinical features, diagnosis, management and prognosis. CASE REPORT: We report a case of promptly diagnosed duodenal GIST in a 61-year-old Egyptian man presented shocked with severe attack of hematemesis and melena. Upper gastroduodenal endoscopy was done and revealed a large ulcerating bleeding mass at first part of duodenum 4 hemo-clips were applied with good hemostasis. An exploratory laparotomy and distal gastrectomy, duodenectomy and gastrojejunostomy were performed. The morphology of the mass combined with immunohistochemistry was consistent with duodenal gastrointestinal stromal tumours (GISTs) of high risk type. The patient is on amatinib one tablet daily and he was well with no evidence of tumor recurrence. CONCLUSION: despite being rare, emergency presentation with sudden severe, life-threatening hemorrhagic shock duodenal GISTs might be a cause of potentially lethal massive combined upper and lower gastrointestinal bleeding which is the key feature of this rare and challenging tumor.


Assuntos
Neoplasias Duodenais , Hemorragia Gastrointestinal , Tumores do Estroma Gastrointestinal , Choque Hemorrágico , Humanos , Tumores do Estroma Gastrointestinal/complicações , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/cirurgia , Masculino , Pessoa de Meia-Idade , Hemorragia Gastrointestinal/etiologia , Neoplasias Duodenais/complicações , Neoplasias Duodenais/cirurgia , Neoplasias Duodenais/patologia , Choque Hemorrágico/etiologia , Melena/etiologia , Hematemese/etiologia , Gastrectomia
4.
ESMO Open ; 9(6): 103477, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38833964

RESUMO

BACKGROUND: Sunitinib is an oral anticancer drug approved for the treatment of among others gastrointestinal stromal tumor (GIST). Previous analyses demonstrated an exposure-response relationship at the standard dose, and minimum target levels of drug exposure have been defined above which better treatment outcomes are observed. Therapeutic drug monitoring (TDM) could be used as a tool to optimize the individual dose, aiming at sunitinib trough concentrations ≥37.5 ng/ml for continuous dosing. Nonetheless, data on the added value of TDM-guided dosing on clinical endpoints are currently lacking. Therefore, we evaluate the effect of TDM in patients with advanced and metastatic GIST treated with sunitinib in terms of efficacy and toxicity. PATIENTS AND METHODS: A TDM-guided cohort was compared to a non-TDM-guided cohort in terms of median progression-free survival (mPFS) and overall survival (mOS). Also, mPFS between patients with and without dose-limiting toxicities (DLTs) was compared. Patients in the prospective cohort were included in two studies on TDM-guided dosing (the DPOG-TDM study and TUNE study). The retrospective cohort consisted of patients from the Dutch GIST Registry who did not receive TDM-guided dosing. RESULTS: In total, 51 and 106 patients were included in the TDM-guided cohort and non-TDM-guided cohort, respectively. No statistical difference in mPFS was observed between these two cohorts (39.4 versus 46.9 weeks, respectively; P = 0.52). Patients who experienced sunitinib-induced DLTs had longer mPFS compared to those who did not (51.9 versus 28.9 weeks, respectively; P = 0.002). CONCLUSIONS: Our results do not support the routine use of TDM-guided dose optimization of sunitinib in patients with advanced/metastatic GIST to improve survival.


Assuntos
Antineoplásicos , Monitoramento de Medicamentos , Tumores do Estroma Gastrointestinal , Sunitinibe , Humanos , Sunitinibe/administração & dosagem , Sunitinibe/uso terapêutico , Sunitinibe/farmacologia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Feminino , Masculino , Pessoa de Meia-Idade , Idoso , Antineoplásicos/uso terapêutico , Antineoplásicos/administração & dosagem , Estudos Retrospectivos , Monitoramento de Medicamentos/métodos , Adulto , Resultado do Tratamento , Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/mortalidade , Relação Dose-Resposta a Droga , Idoso de 80 Anos ou mais , Estudos Prospectivos , Intervalo Livre de Progressão
5.
Folia Med (Plovdiv) ; 66(2): 291-297, 2024 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-38690828

RESUMO

Extra-gastrointestinal stromal tumors arising from the pancreas are extremely rare. To date, just over 30 cases have been described in the world literature. A clinical observation of a 67-year-old patient with dull epigastric pain and a large cystic solid neoplasm instrumentally identified as an extra-gastrointestinal stromal tumor of the head of the pancreas is presented. The volume of surgical intervention consisted of pancreatogastroduodenectomy and right-sided hemicolectomy, since tumor invasion into the transverse mesocolon was detected intraoperatively. The final diagnosis of extra-gastrointestinal stromal sarcoma of the head of the pancreas with invasion into the mesocolon pT4N0M0, stage IIIb was made on the basis of histopathology and immunohistochemistry results.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Pancreáticas , Humanos , Idoso , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Masculino , Pancreaticoduodenectomia
6.
Sci Rep ; 14(1): 11494, 2024 05 20.
Artigo em Inglês | MEDLINE | ID: mdl-38769376

RESUMO

Gastrointestinal stromal tumors (GISTs) predominantly develop in the stomach. While nomogram offer tremendous therapeutic promise, there is yet no ideal nomogram comparison customized specifically for handling categorical data and model selection related gastric GISTs. (1) We selected 5463 patients with gastric GISTs from the SEER Research Plus database spanning from 2000 to 2020; (2) We proposed an advanced missing data imputation algorithm specifically designed for categorical variables; (3) We constructed five Cox nomogram models, each employing distinct methods for the selection and modeling of categorical variables, including Cox (Two-Stage), Lasso-Cox, Ridge-Cox, Elastic Net-Cox, and Cox With Lasso; (4) We conducted a comprehensive comparison of both overall survival (OS) and cancer-specific survival (CSS) tasks at six different time points; (5) To ensure robustness, we performed 50 randomized splits for each task, maintaining a 7:3 ratio between the training and test cohorts with no discernible statistical differences. Among the five models, the Cox (Two-Stage) nomogram contains the fewest features. Notably, at Near-term, Mid-term, and Long-term intervals, the Cox (Two-Stage) model attains the highest Area Under the Curve (AUC), top-1 ratio, and top-3 ratio in both OS and CSS tasks. For the prediction of survival in patients with gastric GISTs, the Cox (Two-Stage) nomogram stands as a simple, stable, and accurate predictive model with substantial promise for clinical application. To enhance the clinical utility and accessibility of our findings, we have deployed the nomogram model online, allowing healthcare professionals and researchers worldwide to access and utilize this predictive tool.


Assuntos
Tumores do Estroma Gastrointestinal , Nomogramas , Programa de SEER , Neoplasias Gástricas , Humanos , Tumores do Estroma Gastrointestinal/mortalidade , Tumores do Estroma Gastrointestinal/patologia , Feminino , Masculino , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Pessoa de Meia-Idade , Prognóstico , Idoso , Modelos de Riscos Proporcionais , Análise de Sobrevida , Algoritmos
7.
Tech Coloproctol ; 28(1): 61, 2024 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-38801613

RESUMO

Gastrointestinal stromal tumours (GISTs) can develop throughout the entire gastrointestinal tract, but these tumours are usually found in the stomach and small intestine. In this case, a rare GIST arising from the anal canal was investigated using high-frequency endoanal ultrasound and external three-dimensional ultrasound with tomographic ultrasound imaging. The endoanal approach revealed the inner structure of the tumour. External ultrasound was used to determine the relationship between the lesion and surrounding tissues. In the limited reports of anal canal GISTs, no other lesions have been correctly diagnosed preoperatively or displayed in detail on imaging. The multilayer structure of the anal sphincter and these lesions can be clearly displayed by a variety of ultrasound imaging methods, which are nonradiative, low-cost and easily accessible. Modern ultrasound has the potential for broad application in anal canal tumour diagnosis and surveillance.


Assuntos
Canal Anal , Neoplasias do Ânus , Endossonografia , Tumores do Estroma Gastrointestinal , Humanos , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Neoplasias do Ânus/diagnóstico por imagem , Neoplasias do Ânus/patologia , Canal Anal/diagnóstico por imagem , Endossonografia/métodos , Imageamento Tridimensional/métodos , Ultrassonografia/métodos , Masculino , Pessoa de Meia-Idade , Feminino , Idoso
8.
Surg Endosc ; 38(6): 3353-3360, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38698259

RESUMO

BACKGROUND AND AIMS: Many studies of gastric gastrointestinal stromal tumors (g-GISTs) following endoscopic resection (ER) have typically focused on tumor size, with most tumors at low risk of aggressiveness after risk stratification. There have been few systematic studies on the oncologic outcomes of intermediate- or high-risk g-GISTs after ER. METHODS: From January 2014 to January 2020, we retrospectively collected patients considered at intermediate- or high-risk of g-GISTs according to the modified NIH consensus classification system. The primary outcome was overall survival (OS). RESULTS: Six hundred and seventy nine (679) consecutive patients were diagnosed with g-GISTs and treated by ER between January 2014 and January 2020 in three hospitals in Shanghai, China. 43 patients (20 males and 23 females) were confirmed at intermediate-or high-risk. The mean size of tumors was 2.23 ± 1.01 cm. The median follow-up period was 62.02 ± 15.34 months, with a range of 28 to 105 months. There were no recurrences or metastases, even among patients having R1 resections. The 5-year OS rate was 97.4% (42/43). CONCLUSION: ER for intermediate- or high-risk gastric small GISTs is a feasible and safe method, which allows for a wait-and-see approach before determining the necessity for imatinib adjuvant or surgical treatment. This approach to g-GISTs does require that patients undergo close follow-up.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/mortalidade , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Neoplasias Gástricas/cirurgia , Neoplasias Gástricas/patologia , Neoplasias Gástricas/mortalidade , Idoso , Adulto , Resultado do Tratamento , Gastroscopia/métodos , Taxa de Sobrevida , China/epidemiologia , Idoso de 80 Anos ou mais , Medição de Risco , Gastrectomia/métodos
9.
J Med Invest ; 71(1.2): 148-153, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38735711

RESUMO

BACKGROUND: Laparoscopic and endoscopic cooperative surgery (LECS) is an effective treatment for gastric gastrointestinal stromal tumors (GISTs). The utility of LECS for gastric GISTs of > 5 cm remains controversial. This study was performed to investigate the feasibility of LECS for gastric GISTs with a tumor diameter of >5 cm. METHODS: We analyzed 43 patients with gastric GISTs who underwent LECS or laparoscopic partial gastrectomy (Lap-Partial Gx). We compared the surgical outcomes of LECS versus Lap-Partial Gx and of LECS for a tumor diameter of > 5 versus ≤ 5 cm. RESULTS: In the comparison of LECS versus Lap-Partial Gx, there were no significant intergroup differences in the operative time or blood loss volume. The morbidity rate was similar between the groups. No postoperative mortality occurred in either group. In the comparison of LECS for a tumor diameter of > 5 versus ≤ 5 cm, there were no significant intergroup differences in operative time, or blood loss volume. The morbidity rate was similar between the > 5-cm and ≤ 5-cm groups (0.0% vs. 4.5%, respectively ; p = 0.56). Additionally, no recurrence or death occurred during follow-up in either group. CONCLUSION: LECS is a feasible option for gastric GISTs with a tumor diameter of > 5 cm. J. Med. Invest. 71 : 148-153, February, 2024.


Assuntos
Estudos de Viabilidade , Tumores do Estroma Gastrointestinal , Laparoscopia , Neoplasias Gástricas , Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Feminino , Masculino , Laparoscopia/métodos , Pessoa de Meia-Idade , Idoso , Neoplasias Gástricas/cirurgia , Neoplasias Gástricas/patologia , Gastrectomia/métodos , Adulto , Estudos Retrospectivos
10.
J Investig Med High Impact Case Rep ; 12: 23247096241253348, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38757744

RESUMO

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the digestive tract and arise from the interstitial cells of Cajal in the mesenteric plexus. These tumors can originate in any part of the GI tract; however, a higher burden has been observed in the stomach and small intestines. Mesenteric GISTs are exceedingly rare, with unique clinicopathological features and a poorer prognosis. Herein, we describe a unique case of a 66-year-old female with a remote history of appendectomy who presented to the emergency room complaining of severe abdominal pain and vomiting. On imaging, the patient was found to have a large inflammatory mass associated with small bowel loops, and the pathology confirmed a mesenteric GIST. The tumor was resected, and the genomic test results confirmed the KIT (exon 11) mutation. Although the tumor had a low mitotic rate, the tumor was large enough to warrant the initiation of adjuvant imatinib mesylate for 36 months with regular bloodwork and imaging.


Assuntos
Abdome Agudo , Tumores do Estroma Gastrointestinal , Mesilato de Imatinib , Mesentério , Humanos , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Feminino , Idoso , Abdome Agudo/etiologia , Mesilato de Imatinib/uso terapêutico , Mesentério/patologia , Proteínas Proto-Oncogênicas c-kit/genética , Tomografia Computadorizada por Raios X , Mutação , Antineoplásicos/uso terapêutico
11.
Sci Rep ; 14(1): 12415, 2024 05 30.
Artigo em Inglês | MEDLINE | ID: mdl-38816560

RESUMO

Gastrointestinal stromal tumors (GISTs) are a rare type of tumor that can develop liver metastasis (LIM), significantly impacting the patient's prognosis. This study aimed to predict LIM in GIST patients by constructing machine learning (ML) algorithms to assist clinicians in the decision-making process for treatment. Retrospective analysis was performed using the Surveillance, Epidemiology, and End Results (SEER) database, and cases from 2010 to 2015 were assigned to the developing sets, while cases from 2016 to 2017 were assigned to the testing set. Missing values were addressed using the multiple imputation technique. Four algorithms were utilized to construct the models, comprising traditional logistic regression (LR) and automated machine learning (AutoML) analysis such as gradient boost machine (GBM), deep neural net (DL), and generalized linear model (GLM). We evaluated the models' performance using LR-based metrics, including the area under the receiver operating characteristic curve (AUC), calibration curve, and decision curve analysis (DCA), as well as AutoML-based metrics, such as feature importance, SHapley Additive exPlanation (SHAP) Plots, and Local Interpretable Model Agnostic Explanation (LIME). A total of 6207 patients were included in this study, with 2683, 1780, and 1744 patients allocated to the training, validation, and test sets, respectively. Among the different models evaluated, the GBM model demonstrated the highest performance in the training, validation, and test cohorts, with respective AUC values of 0.805, 0.780, and 0.795. Furthermore, the GBM model outperformed other AutoML models in terms of accuracy, achieving 0.747, 0.700, and 0.706 in the training, validation, and test cohorts, respectively. Additionally, the study revealed that tumor size and tumor location were the most significant predictors influencing the AutoML model's ability to accurately predict LIM. The AutoML model utilizing the GBM algorithm for GIST patients can effectively predict the risk of LIM and provide clinicians with a reference for developing individualized treatment plans.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Hepáticas , Aprendizado de Máquina , Programa de SEER , Humanos , Tumores do Estroma Gastrointestinal/patologia , Neoplasias Hepáticas/secundário , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Idoso , Prognóstico , Adulto , Algoritmos , Curva ROC , Neoplasias Gastrointestinais/patologia
12.
Acta Oncol ; 63: 288-293, 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38712513

RESUMO

INTRODUCTION: Metastatic gastrointestinal stromal tumour (GIST) is considered incurable, and life-long treatment with tyrosine kinase inhibitors is recommended. We investigated whether selected patients with metastatic GIST may remain in durable remission despite imatinib discontinuation. PATIENTS: In this 1-group, prospective, multicentre phase II trial selected patients with oligometastatic (≤3 metastases) GIST discontinued imatinib treatment. Eligible patients had been treated with imatinib >5 years without progression and had no radiologically detectable metastases after metastasectomy, radiofrequency ablation (RFA) or complete response to imatinib. The primary endpoint was progression-free survival (PFS) 3-years after stopping imatinib. Overall survival (OS) and quality of life (QoL) were secondary endpoints. RESULTS: The trial closed prematurely due to slow accrual. Between January 5, 2017, and June 5, 2019, 13 patients were enrolled, of whom 12 discontinued imatinib. The median follow-up time was 55 months (range, 36 to 69) after study entry. Five (42%) of the 12 eligible patients remained progression free, and seven (58%) progressed with a median time to progression 10 months. Median PFS was 23 months and the estimated 3-year PFS 41%. Six of the seven patients who progressed restarted imatinib, and all six responded. Three-year OS was 100%, and all patients were alive at the time of the study analysis. QoL measured 5 and 11 months after discontinuation of imatinib demonstrated improvement compared to the baseline. INTERPRETATION: A substantial proportion of selected patients with oligometastatic GIST treated with imatinib and metastasis surgery/RFA may remain disease-free for ≥3 years with improved QoL after stopping of imatinib.


Assuntos
Antineoplásicos , Tumores do Estroma Gastrointestinal , Mesilato de Imatinib , Qualidade de Vida , Humanos , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/terapia , Tumores do Estroma Gastrointestinal/mortalidade , Tumores do Estroma Gastrointestinal/cirurgia , Mesilato de Imatinib/uso terapêutico , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Estudos Prospectivos , Antineoplásicos/uso terapêutico , Adulto , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasias Gastrointestinais/mortalidade , Neoplasias Gastrointestinais/terapia , Suspensão de Tratamento , Indução de Remissão , Intervalo Livre de Progressão , Metástase Neoplásica , Idoso de 80 Anos ou mais , Inibidores de Proteínas Quinases/uso terapêutico
13.
Abdom Radiol (NY) ; 49(5): 1716-1733, 2024 05.
Artigo em Inglês | MEDLINE | ID: mdl-38691132

RESUMO

There is a diverse group of non-gastrointestinal stromal tumor (GIST), mesenchymal neoplasms of the gastrointestinal (GI) tract that demonstrate characteristic pathology and histogenesis as well as variable imaging findings and biological behavior. Recent advancements in tumor genetics have unveiled specific abnormalities associated with certain tumors, influencing their molecular pathogenesis, biology, response to treatment, and prognosis. Notably, giant fibrovascular polyps of the esophagus, identified through MDM2 gene amplifications, are now classified as liposarcomas. Some tumors exhibit distinctive patterns of disease distribution. Glomus tumors and plexiform fibromyxomas exhibit a pronounced affinity for the gastric antrum. In contrast, smooth muscle tumors within the GI tract are predominantly found in the esophagus and colorectum, surpassing the incidence of GISTs in these locations. Surgical resection suffices for symptomatic benign tumors; multimodality treatment may be necessary for frank sarcomas. This article aims to elucidate the cross-sectional imaging findings associated with a wide spectrum of these tumors, providing insights that align with their histopathological features.


Assuntos
Neoplasias Gastrointestinais , Humanos , Neoplasias Gastrointestinais/diagnóstico por imagem , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Diagnóstico por Imagem/métodos
15.
Int J Surg ; 110(4): 2151-2161, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38619177

RESUMO

BACKGROUND: The liver is the most common site of metastasis from gastrointestinal stromal tumors (GISTs). The authors aimed to evaluate imatinib (IM) combined with hepatic resection (HR) or other local treatments such as radiofrequency ablation (RFA) and transarterial chemoembolization (TACE), compared to IM monotherapy in long-term survival benefits in patients suffering from GIST liver metastases. METHODS: Our research encompassed 238 patients diagnosed with liver metastases of GISTs from January 2002 to April 2022 at the First Affiliated Hospital of Sun Yat-Sen University. The oncological outcomes of concern included overall survival (OS), progression-free survival (PFS), and liver-specific PFS. RESULTS: Of all 238 patients, 126 were treated with IM alone (IM group), 81 with IM combined with HR (IM+HR group), and 31 with IM combined with RFA/TACE (IM+RFA/TACE group). The median follow-up time was 44.83 months. The median OS in the IM group was 132.60 months and was not reached in either the IM+HR group or the IM+RFA/TACE group. The 10-year OS rate in the IM+HR group was significantly superior to the IM group and the IM+RFA/TACE group (91.9% vs. 61.1% vs. 55.2%, respectively, P =0.015), and the liver-specific PFS ( P =0.642) and PFS ( P =0.369) in the three groups showed a beneficial trend in the combined treatment group. Multivariate analyses showed that age less than or equal to 60 years (HR 0.280, P< 0.001) and IM+HR (HR 0.361, P =0.047) were independently associated with better OS. Achieving no evidence of disease through surgical intervention was independently correlated with enhanced OS (HR 0.099, P =0.034), liver-specific PFS (HR 0.388, P =0.014), and PFS (HR 0.402, P =0.004). CONCLUSIONS: In patients with GIST liver metastases, IM combined with HR might improve OS in selected patients compared with IM alone and IM combined with RFA/TACE. Achieving no evidence of disease status with surgical treatment of patients results in significant prolonging of OS, liver-specific PFS, and PFS.


Assuntos
Antineoplásicos , Tumores do Estroma Gastrointestinal , Hepatectomia , Mesilato de Imatinib , Neoplasias Hepáticas , Humanos , Tumores do Estroma Gastrointestinal/terapia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/secundário , Mesilato de Imatinib/uso terapêutico , Mesilato de Imatinib/administração & dosagem , Masculino , Feminino , Pessoa de Meia-Idade , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/terapia , Neoplasias Hepáticas/tratamento farmacológico , Estudos Retrospectivos , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Adulto , Idoso , Terapia Combinada , Ablação por Radiofrequência , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Neoplasias Gastrointestinais/tratamento farmacológico , Quimioembolização Terapêutica/métodos , Resultado do Tratamento
16.
J Int Med Res ; 52(4): 3000605241240995, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38663880

RESUMO

Intussusception is defined as the invagination of a proximal segment of the bowel into the adjoining or distal segment. In most adults with intussusception, there is a demonstrable lead point with a definite pathologic abnormality. The clinical features of intussusception include chronic intermittent abdominal pain, nausea and vomiting, constipation, and a palpable abdominal mass. The present case report describes a 62-year-old woman with a 2-week history of abdominal pain and 9-day history of vomiting. Clinical, imaging, and histologic evaluations revealed a jejunojejunal intussusception with a gastrointestinal stromal tumor as the lead point. A gastrointestinal stromal tumor should be considered as a possible lead point in adult patients with intussusception. The implication of reducing the intussusception prior to tumor resection requires further evaluation in view of the risk of venous embolism, including direct spread of malignant cells, in cases involving a large polypoid mass with a necrotic surface that extends to the serosa as shown by intraoperative examination. Accordingly, the rationale for adjuvant therapy with imatinib also requires further evaluation.


Assuntos
Tumores do Estroma Gastrointestinal , Intussuscepção , Humanos , Intussuscepção/etiologia , Intussuscepção/cirurgia , Intussuscepção/diagnóstico , Intussuscepção/patologia , Intussuscepção/diagnóstico por imagem , Feminino , Tumores do Estroma Gastrointestinal/complicações , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/diagnóstico , Pessoa de Meia-Idade , Doenças do Jejuno/etiologia , Doenças do Jejuno/cirurgia , Doenças do Jejuno/diagnóstico , Doenças do Jejuno/patologia , Tomografia Computadorizada por Raios X , Dor Abdominal/etiologia
17.
Pathologie (Heidelb) ; 45(3): 223-232, 2024 May.
Artigo em Alemão | MEDLINE | ID: mdl-38587549

RESUMO

For more than 20 years gastrointestinal stromal tumors (GIST) have been a paradigm for a targeted treatment with tyrosine kinase inhibitors. A fundamental prerequisite for a neoadjuvant or adjuvant treatment of localized GIST or an additive treatment of metastatic GIST is the molecular typing of tumors, ideally at the initial diagnosis. In addition, the possibility of a hereditary or syndromic predisposition must be considered because this results in consequences for the treatment and a different follow-up strategy.


Assuntos
Tumores do Estroma Gastrointestinal , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/terapia , Humanos , Inibidores de Proteínas Quinases/uso terapêutico , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/tratamento farmacológico , Antineoplásicos/uso terapêutico , Proteínas Proto-Oncogênicas c-kit/genética
18.
Eur J Surg Oncol ; 50(6): 108310, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38598874

RESUMO

BACKGROUND: Although several prognostic factors in GIST have been well studied such as tumour size, mitotic rate, or localization, the influence of microscopic margins or R1 resection remains controversial. The aim of this study was to evaluate the influence of R1 resection on the prognosis of GIST in a large multicentre retrospective series of patients. METHODS: From 2001 to 2013, 1413 patients who underwent surgery for any site of GIST were identified from 61 European centers. 1098 patients were included, excluding synchronous metastases, concurrent malignancies, R2 resection or GIST recurrence. Tumour rupture (TR) was reclassified according to the Oslo sarcoma classification. Cox proportional hazards ratio and Kaplan-Meier survival estimates were used to analyse 5-year recurrence-free survival (RFS). RESULTS: Of 1098 patients, 38 (3%) underwent R1 resection with a risk of TR of 11%. The 5-year RFS was 89.6% with a median follow-up of 81 months [range: 31.2-152 months]. On univariate analysis, lower RFS was significantly associated with R1 resection [HR = 2.13; p = 0.04], high risk score according to the modified NIH classification, administration of adjuvant therapy [HR = 2.24; p < 0.001] and intraoperative complications [HR = 2.82; p < 0.001]. Only intraoperative complications [HR = 1.79; p = 0.02] and high risk according to the modified NIH classification including the updated definition of TR [HR = 3.43; p = 0.04] remained significant on multivariate analysis. CONCLUSION: This study shows that positive microscopic margins are not an independent predictive factor for RFS in GIST when taking into account the up-dated classification of TR. R1 resection may be considered a reasonable alternative to avoid major functional sequelae and should not lead to reoperation.


Assuntos
Tumores do Estroma Gastrointestinal , Margens de Excisão , Humanos , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Idoso , Prognóstico , Europa (Continente) , Adulto , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/cirurgia , Intervalo Livre de Doença , Idoso de 80 Anos ou mais , Recidiva Local de Neoplasia , Modelos de Riscos Proporcionais , Estimativa de Kaplan-Meier
19.
Appl Immunohistochem Mol Morphol ; 32(5): 229-232, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38584487

RESUMO

Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms with variable behavior characterized by differentiation toward the interstitial cells of Cajal occurring anywhere in the gastrointestinal stromal tract. Frequently, GISTs have fibrous stroma within tumor cell proliferation areas, which is unlike other types of malignant tumors. If this desmoplasia is active, there is a possibility that some sort of transmitter exists between GIST cells and cells related to fibrosis in the tumor cell proliferation areas. Transforming growth factor (TGF)-ß isoforms, particularly TGF-ß1, are critical for fibrosis pathogenesis. TGF-ß1 regulation of myofibroblasts and fibroblasts during fibrosis is well described. The induced fibroblast activation resulting in myofibroblast differentiation has been reported as an important source of collagen, glycoproteins, proteoglycans, and matrix metallopeptidases in wound healing and fibrosis. However, there are a few reports on the relationship between TGF-ß1 and GISTs. This study aims to clarify TGF-ß1 expression in 30 gastric GISTs using immunohistochemistry and reverse transcription polymerase chain reaction (RT-PCR). For comparison, we also enrolled 30 samples of gastric tubular adenocarcinoma (GTAC). We confirmed TGF-ß1 expression (H-score ≥50 points) in 57% of GIST and 13% of GTAC samples, a significant difference between the 2 tumor types ( P =0.001). We examined the TGF-ß1 mRNA expression of 3 representative GIST samples, each having their respective immunostained areas detected by RT-PCR. Finding TGF-ß1 expression may indicate that this cytokine plays a part in the formation of desmoplasia within GIST cell proliferative areas.


Assuntos
Neoplasias Gastrointestinais , Tumores do Estroma Gastrointestinal , Fator de Crescimento Transformador beta1 , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fibrose , Neoplasias Gastrointestinais/metabolismo , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/metabolismo , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/genética , Regulação Neoplásica da Expressão Gênica , Imuno-Histoquímica , Fator de Crescimento Transformador beta1/metabolismo , Fator de Crescimento Transformador beta1/genética
20.
Gan To Kagaku Ryoho ; 51(4): 451-453, 2024 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-38644319

RESUMO

A 87-year-old female was pointed out wall thickness in the upper part of gastric body for examination of anemia. The mass had a contrast effect, some of it protruded outside the wall, and the surrounding lymph nodes were enlarged. Upper endoscopy showed irregular ulcerative lesion with submucosal volume from posterior wall to the greater curvature in the upper part of gastric body. Biopsy was performed, and GIST of stomach was diagnosed. Surgery was performed for the GIST of the stomach. During open surgery, invasion of pancreatic tail was observed, therefore proximal gastrectomy with D1 lymph node dissection and distal pancreatectomy were performed. Pathologically, the tumor measured 95×78×65 mm with mitotic figures(38/50 high-power fields). Immunohistochemical analysis revealed that tumor cells expressed positive results for c-kit, α-SMA and CD34, and negative results for S-100 and desmin on the basis of the histology and immunostaining profile, the tumor was diagnosed as a GIST. The patient was classed as high risk according to Fletcher's risk classification. Tumor invades pancreatic tail, and lymph node metastasis was observed. She was discharged on the postoperative day 27 and alive without tumor recurrence at 6 months after surgery, not undergoing adjuvant chemotherapy.


Assuntos
Gastrectomia , Tumores do Estroma Gastrointestinal , Metástase Linfática , Neoplasias Gástricas , Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Feminino , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia , Neoplasias Gástricas/tratamento farmacológico , Idoso de 80 Anos ou mais , Excisão de Linfonodo
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