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1.
Diagn Cytopathol ; 50(3): E100-E106, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34870907

RESUMO

Glomus tumors make up 1% of stromal tumors of the stomach. Radiologic diagnosis of glomus tumors can be challenging as they share imaging characteristics with other neuroendocrine tumors and gastrointestinal stromal tumors. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) has been reported as a useful method for the evaluation of gastrointestinal lesions. We report two cases of gastric glomus tumors in which EUS-FNA diagnosis was challenging. Cytologically, neoplastic cells were round to oval, uniform, bland appearing epithelioid cells with delicate chromatin and inconspicuous to vague nucleoli. Both samples lacked worrisome features such as high nuclear grade, high mitotic rate, and necrosis. Neoplastic cells were negative for Cam5.2 and AE1/AE3 with focal expression of synaptophysin in one of the cases. A definitive diagnosis was not made based on FNA. Familiarity with glomus tumors in the GI system and procurement of adequate material for cell block allowing the use of immunohistochemistry may allow an accurate preoperative diagnosis.


Assuntos
Tumores do Estroma Gastrointestinal , Tumor Glômico , Neoplasias Gástricas , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Tumores do Estroma Gastrointestinal/patologia , Tumor Glômico/diagnóstico , Tumor Glômico/patologia , Humanos , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/patologia
2.
J Korean Med Sci ; 37(23): e184, 2022 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-35698837

RESUMO

BACKGROUND: No definite guidelines for the management of small esophageal subepithelial tumors (SETs) have been established, because there are limited data and studies on their natural history. We aimed to assess the natural history and propose optimal management strategies for small esophageal SETs. METHODS: Patients diagnosed as esophageal SETs ≤ 30 mm in size between 2003 and 2017 using endoscopic ultrasound (EUS) with a minimal follow-up of 3 months were enrolled, and their esophagogastroduodenoscopy (EGD) and EUS were retrospectively reviewed. RESULTS: Of 275 esophageal SETs in 262 patients, the initial size was < 10 mm, 10-20 mm, and 20-30 mm in 104 (37.8%), 105 (38.2%), and 66 (24.0%) lesions, respectively. Only 22 (8.0%) SETs showed significant changes in size and/or echogenicity and/or morphology at a median of 40 months (range, 4-120 months). Tissues of 6 SETs showing interval changes were obtained using EUS-guided fine needle aspiration biopsy; 1 was identified as a gastrointestinal stromal tumor (GIST) and was surgically resected, while the other 5 were leiomyomas and were regularly observed. Eight SETs showing interval changes were resected surgically or endoscopically without pathological confirmation; 1 was a GIST, 2 were granular cell tumors, and the other 5 were leiomyomas. CONCLUSION: Regular follow-up with EGD or EUS may be necessary for esophageal SETs ≤ 30 mm in size considering that small portion of them has a possibility of malignant potential. When esophageal SETs ≤ 30 mm show significant interval changes, pathological confirmation may precede treatment to avoid unnecessary resection.


Assuntos
Neoplasias Esofágicas , Tumores do Estroma Gastrointestinal , Leiomioma , Endossonografia , Neoplasias Esofágicas/diagnóstico por imagem , Neoplasias Esofágicas/patologia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Humanos , Leiomioma/diagnóstico por imagem , Estudos Retrospectivos
3.
Gulf J Oncolog ; 1(39): 27-30, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35695343

RESUMO

BACKGROUND: Rectal gastrointestinal stromal tumors are rare and optimal treatment is yet to be defined. The aim of this report is to highlight the possible aggressive behavior of four cases of rectal GISTs treated with neoadjuvant imatinib in a tertiary care medical hospital. METHODS: Four cases of rectal GISTs were retrospectively reviewed for patients demographics, clinical presentation, histology, and imatinib therapy. RESULTS: GISTs were common in men. Age ranged to symptoms were nonspecific. All cases were initially considered to have locally unresectable. Patients received preoperative imatinib. Course was unfavorable. 3 patients died of progressive disease, and one from infectious complications. CONCLUSION: Rectal GISTs may be aggressive and resistant to medical treatment. Thus only early diagnosis may offer the best chance of recovery. KEY WORDS: Rectal - gastrointestinal stromal tumor - neoadjuvant imatinib - resistance.


Assuntos
Antineoplásicos , Tumores do Estroma Gastrointestinal , Neoplasias Retais , Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Masculino , Terapia Neoadjuvante , Neoplasias Retais/patologia , Estudos Retrospectivos
4.
BMC Surg ; 22(1): 219, 2022 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-35672812

RESUMO

BACKGROUND: Laparoscopic resection of gastric gastrointestinal stromal tumors (GISTs) is technically feasible and associated with favorable outcomes. We compared the clinical efficacy of hand-assisted laparoscopic surgery (HLS) and total laparoscopic surgery (TLS) for gastric GISTs. METHODS: We retrospectively analyzed the clinical data of 69 consecutive patients diagnosed with a gastric GIST in a tertiary referral teaching hospital from December 2016 to December 2020. Surgical outcomes were compared between two groups. RESULTS: Fifty-three patients (TLS group: n = 36; HLS group: n = 17) were included. The mean age was 56.9 and 58.1 years in the TLS and HLS groups, respectively. The maximum tumor margin was significantly shorter in the HLS group than in the TLS group (2.3 ± 0.9. vs. 3.0 ± 0.8 cm; P = 0.004). The operative time of the HLS group was significantly shorter than that of the TLS group (70.6 ± 19.1 min vs. 134.4 ± 53.7 min; P < 0.001). The HLS group had less intraoperative blood loss, a shorter time to first flatus, and a shorter time to fluid diet than the TLS group (P < 0.05). No significant difference was found between the groups in the incidence or severity of complications within 30 days after surgery. Recurrence or metastasis occurred in four cases (HLS group; n = 1; TLS group; n = 3). CONCLUSIONS: This study demonstrated that compared with TLS, HLS for gastric GISTs has the advantages of simpler operation, shorter operative time, and faster postoperative recovery.


Assuntos
Tumores do Estroma Gastrointestinal , Laparoscopia Assistida com a Mão , Laparoscopia , Neoplasias Gástricas , Gastrectomia , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Tempo de Internação , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia , Resultado do Tratamento
6.
BMC Surg ; 22(1): 202, 2022 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-35597932

RESUMO

BACKGROUND: Gastrointestinal stromal tumors (GIST) are rare abdominal tumors. Pretreatment biopsies may be used to diagnose a GIST and enable tailored treatment. Some experts are skeptical about biopsies because they fear tumor cell seeding. The objective of this study was to determine if pretreatment biopsy is associated with increased tumor recurrence. METHODS: We performed a systematic literature search and included studies assessing the oncological outcome of GIST patients who underwent a pre-treatment core needle biopsy or fine needle aspiration. We assessed methodological quality with the Newcastle-Ottawa-Scale for non-randomized studies. This review was registered in the PROSPERO database (CRD42021170290). RESULTS: Three non-randomized studies and eight case reports comprising 350 patients were eligible for inclusion. No prospective study designed to answer the review question was found. One case of needle tract seeding after percutaneous core needle biopsy of GIST was reported. None of the studies reported an increased rate of abdominal recurrence in patients with pretreatment biopsy. CONCLUSIONS: The existing evidence does not indicate a relevant risk of needle tract seeding or abdominal recurrence after pre-treatment biopsy of GIST. Biopsy can safely be done to differentiate GIST from other tumors and to select the most appropriate treatment.


Assuntos
Tumores do Estroma Gastrointestinal , Abdome/patologia , Biópsia por Agulha Fina , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Estudos Prospectivos
7.
J Vis Exp ; (183)2022 05 02.
Artigo em Inglês | MEDLINE | ID: mdl-35575516

RESUMO

Gastrointestinal stromal tumor (GIST) is the most common human sarcoma and is typically driven by a single mutation in the KIT receptor. Across tumor types, numerous mouse models have been developed in order to investigate the next generation of cancer therapies. However, in GIST, most in vivo studies use xenograft mouse models which have inherent limitations. Here, we describe an immunocompetent, genetically engineered mouse model of gastrointestinal stromal tumor harboring a KitV558Δ/+ mutation. In this model, mutant KIT, the oncogene responsible for most GISTs, is driven by its endogenous promoter leading to a GIST which mimics the histological appearance and immune infiltrate seen in human GISTs. Furthermore, this model has been used successfully to investigate both targeted molecular and immune therapies. Here, we describe the breeding and maintenance of a KitV558Δ/+ mouse colony. Additionally, this paper details the treatment and procurement of GIST, draining mesenteric lymph node, and adjacent cecum in KitV558Δ/+ mice, as well as sample preparation for molecular and immunologic analyses.


Assuntos
Tumores do Estroma Gastrointestinal , Animais , Modelos Animais de Doenças , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Humanos , Técnicas Imunológicas , Camundongos , Mutação , Proteínas Proto-Oncogênicas c-kit/genética
8.
BJS Open ; 6(3)2022 05 02.
Artigo em Inglês | MEDLINE | ID: mdl-35594280

RESUMO

BACKGROUND: Rectal gastrointestinal stromal tumours (GISTs) are rare and treated mainly by radical surgery. Although the importance of perioperative imatinib has been recognized, there are few reports on its outcomes. METHOD: Consecutive patients diagnosed with rectal GISTs between July 2008 and February 2021 were identified from a prospective database. Effects of perioperative imatinib were investigated, and surgical and survival outcomes were compared between neoadjuvant imatinib and upfront surgery. RESULTS: 34 patients meeting the inclusion criteria were identified. Compared with upfront surgery (n = 11), the neoadjuvant imatinib group (n = 23) had significantly larger tumours (median size 8.3 versus 2.5 cm; P = 0.01) and included a significantly greater proportion of high-risk patients according to the modified Fletcher classification (20/23 (87.0%) versus 6/11 (54.5%); P = 0.02). Comparing the operation planned based on imaging before neoadjuvant imatinib and the operation performed, there was an increase in sphincter-preserving surgery (4/23 (17.4%) to 11/23 (47.8%); P = 0.02), abdominoperineal resection 11/23 (47.8%) reduced to 7/23 (30.4%); P = 0.13) and total pelvic exenteration reduced from 8/23 (34.8%) to 5/23 (21.7%); P = 0.01). Tumours were downsized by a median of 30 per cent (range 0 per cent to -56 per cent; P = 0.01). During follow-up (median 42, range 5-131 months), there was no postoperative recurrence in 29 patients who received perioperative imatinib. One of the five patients who underwent surgery without neoadjuvant or adjuvant imatinib developed local recurrence. CONCLUSION: Treatment with imatinib for rectal GISTs seems to improve outcomes, and neoadjuvant imatinib increases the rate of sphincter-preserving surgery.


Assuntos
Antineoplásicos , Tumores do Estroma Gastrointestinal , Neoplasias Retais , Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Mesilato de Imatinib/uso terapêutico , Neoplasias Retais/tratamento farmacológico , Neoplasias Retais/cirurgia , Estudos Retrospectivos
9.
BMC Cancer ; 22(1): 511, 2022 May 06.
Artigo em Inglês | MEDLINE | ID: mdl-35524239

RESUMO

BACKGROUND: Acquired resistance to approved tyrosine kinase inhibitors limits their clinical use in patients with gastrointestinal stromal tumor (GIST). This study investigated the safety, tolerability and efficacy of alpelisib, a phosphatidylinositol 3-kinase inhibitor, used in combination with imatinib in patients with advanced GIST who had failed prior therapy with both imatinib and sunitinib. METHODS: This phase 1b, multicenter, open-label study consisted of 2 phases: dose escalation and dose expansion. Dose escalation involved 200 mg once daily (QD) alpelisib, initially, followed by 250 and 350 mg. These were combined with 400 mg QD imatinib until maximum tolerated dose (MTD) and/or a recommended phase 2 dose (RP2D) of alpelisib in combination with imatinib was determined. This MTD/RP2D dose was tested to evaluate the clinical activity of this combination in dose expansion. RESULTS: Fifty-six patients were enrolled, 21 and 35 in the dose escalation and expansion phases, respectively. The MTD of alpelisib given with imatinib was determined as 350 mg QD. Combination treatment showed partial response in 1 (2.9%) and stable disease in 15 (42.9%) patients. Median progression-free survival was 2 months (95% CI 1.8-4.6). Overall, 92.9% patients had adverse events (AEs) while 46.4% had grade 3/4 AEs, hyperglycemia being the most common (23.2%). CONCLUSIONS: The MTD of alpelisib was estimated as 350 mg QD when used in combination with imatinib 400 mg QD after oral administration in patients with advanced GIST. The safety and tolerability profile of this combination was acceptable; however, the combination did not demonstrate sufficient clinical activity to justify additional clinical testing. TRIAL REGISTRATION: ClinicalTrials.gov NCT01735968 (date of initial registration 28/11/2012).


Assuntos
Tumores do Estroma Gastrointestinal , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Tiazóis , Resultado do Tratamento
10.
Sci Rep ; 12(1): 8275, 2022 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-35585158

RESUMO

Although KIT-mutant GISTs can be effectively treated with tyrosine kinase inhibitors (TKIs), many patients develop resistance to imatinib mesylate (IM) as well as the FDA-approved later-line agents sunitinib, regorafenib and ripretinib. Resistance mechanisms mainly involve secondary mutations in the KIT receptor tyrosine kinase gene indicating continued dependency on the KIT signaling pathway. The fact that the type of secondary mutation confers either sensitivity or resistance towards TKIs and the notion that secondary mutations exhibit intra- and intertumoral heterogeneity complicates the optimal choice of treatment in the imatinib-resistant setting. Therefore, new strategies that target KIT independently of its underlying mutations are urgently needed. Homoharringtonine (HHT) is a first-in-class inhibitor of protein biosynthesis and is FDA-approved for the treatment of chronic myeloid leukemia (CML) that is resistant to at least two TKIs. HHT has also shown activity in KIT-mutant mastocytosis models, which are intrinsically resistant to imatinib and most other TKIs. We hypothesized that HHT could be effective in GIST through downregulation of KIT expression and subsequent decrease of KIT activation and downstream signaling. Testing several GIST cell line models, HHT led to a significant reduction in nascent protein synthesis and was highly effective in the nanomolar range in IM-sensitive and IM-resistant GIST cell lines. HHT treatment resulted in a rapid and complete abolishment of KIT expression and activation, while KIT mRNA levels were minimally affected. The response to HHT involved induction of apoptosis as well as cell cycle arrest. The antitumor activity of HHT was confirmed in a GIST xenograft model. Taken together, inhibition of protein biosynthesis is a promising strategy to overcome TKI resistance in GIST.


Assuntos
Antineoplásicos , Neoplasias Gastrointestinais , Tumores do Estroma Gastrointestinal , Antineoplásicos/farmacologia , Antineoplásicos/uso terapêutico , Resistencia a Medicamentos Antineoplásicos/genética , Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Mepesuccinato de Omacetaxina/farmacologia , Humanos , Mesilato de Imatinib/farmacologia , Mesilato de Imatinib/uso terapêutico , Mutação , Inibidores de Proteínas Quinases/farmacologia , Inibidores de Proteínas Quinases/uso terapêutico , Proteínas Proto-Oncogênicas c-kit/metabolismo
11.
Korean J Gastroenterol ; 79(4): 177-181, 2022 04 25.
Artigo em Inglês | MEDLINE | ID: mdl-35473776

RESUMO

Malignant gastrointestinal stromal tumors (GISTs) are rare neoplasms originating from the gastrointestinal tract that rarely occur in patients below 40 years of age. To our knowledge, there have been no previous reports of satellite and metastatic nodules in GIST. We present a case of a young patient with a huge malignant gastric GIST accompanied by spontaneous bleeding and satellite and metastatic nodules, successfully treated surgically, without preoperative chemotherapy administration. A 28-year-old man was admitted to Haeundae Paik Hospital with melena. A huge bulging gastric mass with ulceration and bleeding was observed on endoscopy. A subepithelial lesion on the stomach body, abutting the pancreatic body and tail, with regional lymph node enlargement was confirmed by EUS and CT. Radical total gastrectomy was performed, the invasion surrounding the pancreatic tail and spleen were surgically dissected, and enlarged lymph nodes around the celiac trunk and the common hepatic artery were removed. The pathology results showed a malignant GIST with two satellite nodules and a metastatic tumor nodule at the left paracardial lymph node site. After complete resection of the malignant GIST, adjuvant chemotherapy with imatinib was initiated. Follow-up CT and endoscopy performed 6 months after surgery confirmed no recurrence of the disease.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Adulto , Gastrectomia , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Linfonodos/patologia , Masculino , Neoplasias Gástricas/patologia
12.
Int J Clin Oncol ; 27(7): 1164-1172, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35435530

RESUMO

BACKGROUND: Regorafenib is an oral multi-kinase inhibitor that has been established as third-line treatment for patients after the failure of imatinib and sunitinib. However, since clinical data of regorafenib in the Japanese population are still lacking, the management of regorafenib is mainly based on the clinical experience of each oncologist. The aim of this study was to evaluate the efficacy and safety of regorafenib in a Japanese population. METHODS: Thirty-three patients treated with regorafenib for metastatic and recurrent gastrointestinal stromal tumors were retrospectively enrolled. This study investigated the anti-tumor effect, including overall survival, progression-free survival, and safety, which was evaluated based on the incidence of adverse events. RESULTS: The median overall survival of patients treated with regorafenib was 23.8 months and the 1-year overall survival rate was 80.0%, the median progression-free survival was 7.1 months and the 1-year progression-free survival rate was 40.2%. The responses to regorafenib were partial response in 3 cases (9.1%), stable disease in 17 (51.5%), progressive disease in 10 (30.3%), and non-evaluable in 3 (9.1%). The disease control rate was 54.0%. Treatment-related adverse events were reported in all patients, with the most common being hand-foot syndrome (72.7%), followed by liver damage (36.4%) and diarrhea (27.3%), and six patients (20.0%) were discontinued due to adverse events. CONCLUSION: This is the first report of Japanese patients with gastrointestinal stromal tumors treated with regorafenib. Regorafenib showed efficacy and a manageable safety profile in Japanese patients with advanced gastrointestinal stromal tumors, which was comparable with previous studies.


Assuntos
Tumores do Estroma Gastrointestinal , Resistencia a Medicamentos Antineoplásicos , Tumores do Estroma Gastrointestinal/patologia , Humanos , Indóis/uso terapêutico , Japão , Recidiva Local de Neoplasia/tratamento farmacológico , Compostos de Fenilureia , Piridinas , Pirróis/uso terapêutico , Estudos Retrospectivos
13.
Zhonghua Yi Xue Za Zhi ; 102(13): 954-960, 2022 Apr 05.
Artigo em Chinês | MEDLINE | ID: mdl-35385968

RESUMO

Objective: To investigate the feasibility of multi-slice spiral CT(MSCT) imaging features of gastric stromal tumor (GST) in predicting pathological NIH risk classification, providing imaging basis for patients with GST before treatment. Methods: The clinical and CT imaging data of 504 patients(506 GST lesions), 259males and 245 females, aged from 13 to 85(60±11) years, with GST confirmed by surgery and pathology collected in the Zhongshan Hospital Affiliated to Fudan University and the Affiliated TCM Hospital of Southwest Medical University. According to pathological NIH risk classification, 506 lesions were divided into low risk group (very low and low risk degree, 277 lesions) and high risk group (medium and high risk degree, 229 lesions).Clinical data and imaging characteristics were compared between two groups. Multivariate logistic regression analysis was performed to screen out independent risk factors for statistically significant imaging indicators. Receiver operating curve (ROC) was used to evaluate the predictive value of tumor length for risk classification. Resulst: Between low risk group and high risk group,there were significant differences in gender(male/female:131/146 vs 129/100), gastrointestinal bleeding(present/absent:39/238 vs 59/170), morphology(regular/Irregular:218/59 vs 95/134), calcification(present/absent:36/241 vs 53/176), degree of necrosis(0°/Ⅰ°/Ⅱ°/Ⅲ°:197/61/16/3 vs 58/98/32/41), ulceration(present/absent:32/245 vs 94/135), growth pattern(endophytic/exophytic/mixed:102/105/70 vs 44/98/87), tumor location(fundus/cardia/body/angle/antrum:98/7/135/12/25 vs 98/6/114/5/6), feeding artery(present/absent:32/245 vs 104/125), vascular enhancement(present/absent:19/258 vs 88/141), effusion of around the disease(present/absent:0/277 vs 13/216), positive sign of fat around the disease(present/absent:0/277 vs 30/199),maximum long diameter[2.82(2.04,3.80) cm vs 5.93(4.06,8.29) cm] and short diameter [2.31(1.60,2.88) cm vs 4.40(3.21,6.37) cm]of tumor(all P<0.05).The maximum long diameter of tumor (OR=2.08,95%CI:1.35-3.20) and ulceration positive(OR=2.01,95%CI:1.03-3.92) were independent risk factors of risk classification(all P<0.05).Gastric antrum was used as the reference for tumor location, gastric fundus(OR=7.77,95%CI:2.00-30.24) and gastric body(OR=3.93,95%CI:1.03-15.01) were independent risk factors of risk classification(all P<0.05).The area under curve(AUC) of the maximum long diameter of tumor for predicting risk classification was 0.87, and the optimal critical value, sensitivity and specificity were 4.98cm, 62.9% and 95.3% respectively. Conclusions: MSCT image features of GST had certain characteristics. MSCT has certain predictive value for pathological NIH risk classification of GST, which can provide certain imaging basis for patients before treatment.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Cárdia/patologia , Estudos de Viabilidade , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , National Institutes of Health (U.S.) , Estudos Retrospectivos , Neoplasias Gástricas/cirurgia , Tomografia Computadorizada Espiral , Estados Unidos , Adulto Jovem
14.
Acta Oncol ; 61(6): 663-668, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35481400

RESUMO

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are malignant mesenchymal tumors arising in the gastrointestinal tract. Their systemic treatment is based on the use of tyrosine kinase inhibitors (TKIs) with imatinib, sunitinib, and regorafenib being the preferred agents. Assessment of tumor response to TKI treatment in GISTs is traditionally done according the Response Evaluation Criteria in Solid Tumors (RECIST), while Choi criteria have also been proposed as alternative tool assessing both volumetric and density changes on computer tomography (CT) scans. EORTC STBSG 1317 'CaboGIST' was a single-arm prospective Phase 2 trial which met its primary endpoint, as 60% of patients previously treated with imatinib and sunitinib were progression-free at 12 weeks (95% CI 45-74%) based on local RECIST assessment. MATERIALS AND METHODS: We report here an exploratory analysis of local versus central RECIST version 1.1 assessment and a comparison of RECIST version 1.1 versus Choi criteria. RESULTS: Comparisons between local and central RECIST version 1.1 at week 12 revealed discrepancies in 17/43 evaluable cases (39.5%). When comparing Choi with local and central RECIST version 1.1, discrepancies were observed in 27/43 (62.8%) and 21/43 (48.8%) cases, respectively. A total of 68% of evaluable patients were progression-free and alive at week 12 based on local RECIST, 84% according to central RECIST analysis and 81% when applying Choi criteria. Central assessment upgraded the treatment response both with RECIST version 1.1 and Choi. CONCLUSIONS: The results of this exploratory analysis support the conclusion that cabozantinib is active in patients with metastatic or recurrent GIST after treatment with imatinib and sunitinib and confirm once again the limitations of RECIST to capture response to TKI in GIST, and the importance to include density changes in the response evaluation in this setting. Clinical trial number: EORTC 1317, NCT02216578.


Assuntos
Antineoplásicos , Tumores do Estroma Gastrointestinal , Anilidas , Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Estudos Prospectivos , Piridinas , Sunitinibe/uso terapêutico , Resultado do Tratamento
15.
BMC Gastroenterol ; 22(1): 182, 2022 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-35410177

RESUMO

BACKGROUND AND OBJECTIVES: Up till now, there are still controversies about the specific indication of endoscopic resection for small gastric subepithelial tumors (gSETs) originating from muscularis propria. We aimed to investigate the safety of endoscopic resection and postoperative pathology analysis. METHOD: The patients with primary small gSETs originating from muscularis propria, treated by endoscopic resection in the endoscopic center of Shengjing Hospital between January, 2011 and September, 2019 were enrolled. The complete resection rate, adverse events and clinicopathological features were recorded. RESULT: A total of 936 patients with 972 gastric SETs ≤ 2 cm originating from muscularis propria were included in our study. All the lesions were successfully treated by endoscopic resection. Nearly half of lesions were proved to be gastrointestinal stromal tumor (GIST) [n = 411 (42.3%)] according to postoperative pathology. All the objects were further subdivided into 2 groups, ≤ 1 cm, > 1 and ≤ 2 cm gSETs. The risk of gastric GIST of intermediate/high risk in the group (> 1 and ≤ 2 cm gSETs) is 8.41 times as that of gastric GIST in the group (the size of gastric ≤ 1 cm gSETs) (P < 0.05). CONCLUSION: Endoscopic resection is a safe and effective treatment for small gSETs. gSETs (1-2 cm) is more risky than gSETs (≤ 1 cm) and should be resected. This should be evaluated by further studies.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Mucosa Gástrica/patologia , Mucosa Gástrica/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Gastroscopia , Humanos , Estudos Retrospectivos , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia , Resultado do Tratamento
16.
J Med Case Rep ; 16(1): 174, 2022 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-35490251

RESUMO

BACKGROUND: Neurofibromatosis type 1 is an inherited cancer predisposition syndrome that is caused by a mutation in the NF1 gene that encodes neurofibromin. Patients with neurofibromatosis type 1 have a higher risk of gastrointestinal stromal tumor. This study reports the case of a patient with gastrointestinal stromal tumor who was later diagnosed to have neurofibromatosis type 1 and, unlike usual features, had some uncommon features such as occurrence at an early age and unusual site of origin. CASE: We report the case of a 29-year-old Indian female diagnosed to have gastrointestinal stromal tumor originating from the greater curvature of the stomach. Gastrointestinal stromal tumor was wild type, negative for c-kit and platelet-derived growth factor receptor, and had an aggressive clinical course not responding to oral tyrosine kinase inhibitors. On later evaluation, we found that the patient had germline mutation in NF1. This case has some unusual features compared with gastrointestinal stromal tumor cases reported in neurofibromatosis type 1. Firstly, the age of onset for gastrointestinal stromal tumor in neurofibromatosis type 1 is earlier in our case compared with previous cases reported in literature. Secondly, the site of occurrence is in the stomach, without involving other parts of the intestine. Gastrointestinal stromal tumor in neurofibromatosis type 1 is usually multifocal, and small intestine is the common site of occurrence. When occurring in the stomach, it is usually associated with other lesions in the small intestine. Lastly, the clinical course is aggressive compared with previous case reports and series. CONCLUSION: Our patient had germline NF1 mutation and cutaneous stigmata of neurofibromatosis. Our patient had unicentric gastrointestinal stromal tumor occurring at younger age and involving greater curvature of the stomach, with spindle cell type histology and high-risk features. If gastrointestinal stromal tumor occurs at young age, we should look into neurocutaneous markers.


Assuntos
Tumores do Estroma Gastrointestinal , Neurofibromatose 1 , Adulto , Feminino , Tumores do Estroma Gastrointestinal/patologia , Mutação em Linhagem Germinativa , Humanos , Mutação , Neurofibromatose 1/complicações , Neurofibromatose 1/patologia , Proteínas Proto-Oncogênicas c-kit/genética
17.
Nutrition ; 98: 111636, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35452975

RESUMO

OBJECTIVES: The aim of this study was to investigate the predictive effects of skeletal muscle mass (SMM) depletion on relapse risk in patients who had undergone complete surgical resection for primary resectable gastrointestinal stromal tumors (GISTs). METHODS: This retrospective study comprised 445 enrolled patients with primary resectable GISTs who had undergone surgical treatment between January 2013 and January 2021. The lumbar skeletal muscle index (SMI) was assessed using abdominal computed tomography images taken within 7 d preoperatively. Univariate and multivariate Cox regression analyses were performed to identify independent risk factors for nomogram construction. Predictive accuracy and discriminative ability were measured using the concordance index (C-index). RESULTS: Three- and 5-y relapse-free survival (RFS) rates for patients in the low SMI group were significantly worse than those in the high SMI group (81.3 and 75.4% versus 92.3 and 91.6%, respectively; P < 0.001). In stratification analysis using modified National Institutes of Health criteria, high-risk patients with low SMI showed significantly shorter RFS (P = 0.001). Multivariate analysis indicated that tumor size, tumor location, mitotic rates, the platelet-to-lymphocyte ratio, the prognostic nutritional index, and SMM depletion were independent prognostic factors for RFS (P < 0.05). These six variables were selected for nomogram construction, which showed superior discrimination with a C-index of 0.82. CONCLUSIONS: There was a significant association between preoperative SMM depletion and a high risk for relapse in patients who had undergone complete resection for primary resectable GISTs, especially in patients with high-risk GIST. Our simple, practical, novel nomogram intuitively predicted RFS in these patients.


Assuntos
Tumores do Estroma Gastrointestinal , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Músculo Esquelético/patologia , Recidiva Local de Neoplasia , Prognóstico , Recidiva , Estudos Retrospectivos
19.
Abdom Radiol (NY) ; 47(6): 1988-2003, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35347384

RESUMO

Mesenchymal tumors of the stomach are uncommon, with gastrointestinal stromal tumor (GIST) being the most common among them. Majority of the tumors may arise from cells of Cajal, smooth muscle cells, neural cells, totipotent stem cells, adipocytes or fibroblasts. Imaging plays an important role not only in staging but also in characterizing these tumors. Many of these tumors have characteristic imaging features. GISTs usually present as large cavitating and necrotic tumors with exophytic component. Presence of fat tissue within the tumor suggests a lipoma or a teratoma, early phase hyperenhancement indicates glomus tumor and hemangioma, and delayed contrast enhancement is seen in schwannoma. Their differentiation from epithelial tumors like carcinoma and neuroendocrine tumors is often possible based on the location (mesenchymal tumors are intramural), spread, morphological appearance and enhancement patterns. However, overlapping features exist between these tumors with imaging often being only suggestive. A biopsy is necessary for a definitive diagnosis in many cases.


Assuntos
Neoplasias Gastrointestinais , Tumores do Estroma Gastrointestinal , Tumor Glômico , Neoplasias Gástricas , Diagnóstico por Imagem , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Humanos , Neoplasias Gástricas/diagnóstico por imagem , Neoplasias Gástricas/patologia
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