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1.
Medicine (Baltimore) ; 99(41): e22497, 2020 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-33031286

RESUMO

RATIONALE: Paragangliomas (PGLs) are rare neuroendocrine tumors that are strongly influenced by genetics, and succinate dehydrogenase-deficient PGLs appear to constitute one of the most important categories. Interestingly, somatic PGLs only possess genomic alterations involving the SDHB and SDHD subunits, and no SDHA alterations have been described. Here, we are presenting the clinical and genetic analyses of 2 cases with the first somatic SDHA variant identified in PGLs. PATIENT CONCERNS: Here, we reported 2 family members with the diagnosis of PGL. Patient 1 is a 55-year-old woman with a functionally perigastric PGL that co-occurred with a gastric gastrointestinal stromal tumor (GIST), and patient 2 is a 43-year-old woman with a nonfunctionally pericardial PGL, who was the younger sister of the first patient. DIAGNOSES: Imaging surveys of the 2 cases depicted the presence of a perigastric and a pericardial mass, respectively. A diagnosis of paragangliomas was established by immunohistochemistry (IHC). INTERVENTIONS: Both patients underwent single-stage resection of the lesion after preoperative oral α-adrenoceptor therapy for 2 weeks. We later performed comprehensive genomic profiling on the tumor samples, including PGL and GIST from patient 1 and PGL from patient 2, and searched for novel actionable mutations, including in all succinate dehydrogenase subunits, as the IHC results were negative for SDHB. OUTCOMES: Both patients had an uneventful recovery after surgery and the sequencing showed a novel somatic variant in the SDHA gene on chromosome 5q11 (c.1945_1946delTT). Regular follow-up with biochemical testing and image studies showed no evidence of recurrence after a year for patient 1 and 6 years for patient 2. LESSONS: PGLs often lead to considerable diagnostic difficulty due to their multiple anatomical locations and variable symptoms, as presented by our cases. The comprehensive use of images and plasma/urine catecholamine measurement can aid the diagnosis of PGLs. In addition, our findings also demonstrate the usefulness and importance of genetic analysis of SDHA mutations in patients exhibiting SDHB IHC-negative PGL. Additional studies utilizing comprehensive genomic profiling are needed to identify the group of PGLs harboring this SDHA genomic alteration.


Assuntos
Complexo II de Transporte de Elétrons/genética , Tumores do Estroma Gastrointestinal/genética , Neoplasias Primárias Múltiplas/genética , Paraganglioma Extrassuprarrenal/genética , Neoplasias Gástricas/genética , Adulto , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Testes Genéticos , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Mutação , Paraganglioma Extrassuprarrenal/diagnóstico , Paraganglioma Extrassuprarrenal/patologia , Irmãos , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/patologia
2.
Medicine (Baltimore) ; 99(40): e22493, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33019445

RESUMO

RATIONALE: Gastrointestinal stromal tumors that present outside the gastrointestinal tract are known for extra-gastrointestinal stromal tumors (EGISTs) and they share the same morphological and immunohistochemical characteristics with gastrointestinal stromal tumors. Here we report a rare case of diffuse primary EGIST arising at peritoneum. PATIENT CONCERNS: A 57-year-old male presented to the hospital with abdominal pain and right lower abdominal tenderness. DIAGNOSIS: The core needle puncture biopsy showed epithelial-like cells and the nuclei were ovoid and focally elongated. Immunohistochemical examination was consistent with a primary EGIST of the peritoneum. INTERVENTIONS: The patient was treated with Imatinib mesylate. OUTCOMES: Five months later, there is no complication resulting from treatment. The follow-up abdominal contrast-enhanced CT showed the lesion was significantly decreased in size, and was evaluated as partial response. The patient continued the treatment with Imatinib as prescribed by the oncologist. LESSONS: EGISTs are rare and should be considered in the differential diagnosis of the peritoneal tumors and immunohistochemistry helps to confirm the diagnosis. Further study with longer follow-up is desired to characterize these uncommon tumors.


Assuntos
Tumores do Estroma Gastrointestinal/patologia , Neoplasias Peritoneais/patologia , Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Humanos , Mesilato de Imatinib/uso terapêutico , Masculino , Pessoa de Meia-Idade , Neoplasias Peritoneais/diagnóstico , Neoplasias Peritoneais/tratamento farmacológico
4.
Zhonghua Wei Chang Wai Ke Za Zhi ; 23(9): 861-865, 2020 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-32927510

RESUMO

The therapeutic choice of duodenal gastrointestinal stromal tumor (GIST) has always been the focus of surgeons because of its special anatomy location. So far, surgery is the preferable treatment for primary duodenal GIST, including pancreaticoduodenectomy (PD) and local resection (LR). Researches reveal that the prognosis of duodenal GIST is determined by the pathologic factors of the tumor itself, and is not significantly associated with the surgical procedure. The intervention with targeted drugs such as imatinib has given the duodenal GIST more opportunities for LR. Meanwhile, the technique development of the laparoscopy combined with endoscopic surgery and robotic surgery ensures the steps of minimally invasive treatment for duodenal GIST into a new era.


Assuntos
Neoplasias Duodenais/cirurgia , Duodeno/cirurgia , Tumores do Estroma Gastrointestinal/cirurgia , Pancreaticoduodenectomia/métodos , Antineoplásicos/uso terapêutico , Terapia Combinada , Neoplasias Duodenais/patologia , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/uso terapêutico , Procedimentos Cirúrgicos Minimamente Invasivos , Prognóstico , Procedimentos Cirúrgicos Robóticos
5.
Zhonghua Wei Chang Wai Ke Za Zhi ; 23(9): 888-895, 2020 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-32927514

RESUMO

Objective: To compare the efficacy between laparoscopy and open surgery for gastric gastrointestinal stromal tumor (GIST) larger than 2 cm. Methods: A multicenter retrospective cohort study was performed. Inclusion criteria: long diameter of primary gastric GIST > 2 cm; undergoing laparoscopy or open surgery; diagnosis confirmed by postoperative pathology without distant metastasis; without preoperative targeted therapy. Clinicopathological data of 857 gastric GIST patients, including 320 in PLA General Hospital, 284 in Shanghai Renji Hospital, 175 in Wuhan Union Hospital and 78 in Tianjin Cancer Hospital, from January 2010 to May 2017 were retrospectively collected. There were 418 males and 439 females, mainly aged between 50 and 70 years old. Among 857 patients, 413 were in the laparoscopy group and 444 in the open group. The nearest neighbor matching of propensity score matching method was conducted with 1:1 matching based on tumor location and size between laparoscopy and open group to obtain samples of covariate equilibrium, and the caliper value was 0.04. The t test, χ(2) test and Wilcoxon rank test were used to compare short-term efficacy, and the Kaplan-Meier curve and log rank test were applied to compare long-term outcomes between the two groups. Results: After propensity score matching, laparoscopy group and open group both enrolled 293 cases. The baseline data, including age, gender, tumor location, tumor long diameter, NIH classification, etc. were not significantly different between the two groups (all P>0.05). Compared with the open group, the laparoscopy group had less intraoperative blood loss [<100 ml: 2.9% (155/293) vs. 36.2% (106/293), Z=-12.857, P<0.001], shorter time to postoperative feeding [(4.0±0.2) days vs. (5.3±0.9) days, t=1.505, P=0.003] and to the removal of drainage tube [(4.8±1.0) days vs. (6.5±1.0) days, t=1.847, P=0.008], and shorter postoperative hospital stay [(8.6±0.3) days vs. (10.5±0.3) days, t=4.235, P<0.001]. Subgroups analysis according to anatomical location: (1) Gastric cardia and pylorus: there were no statistically significant differences in perioperative parameters between the two groups (all P>0.05). (2) Stomach base: feeding time after surgery [(4.0±0.2) days vs. (4.5±0.2) days, t=0.512, P=0.038], drainage tube removal time [(5.1±0.4) days vs. (6.4±0.6) days, t=0.517, P=0.044], postoperative hospital stay [(8.0±0.5) days vs. (11.1±0.9) days, t=0.500, P=0.002] were all significantly shorter in the laparoscopy group as compared to the open group, while the differences in other perioperative parameters were not statistically significant (all P>0.05). (3) Lesser curvature of the stomach: the laparoscopy group had less intraoperative blood loss [<100 ml ratio: 58.1% (43/74) vs. 33.7% (25/74), Z=7.632, P=0.034], shorter gastric tube removal time [(2.7±0.2) days vs. (3.2±0.3) days, t=0.503, P=0.007], earlier postoperative passage of gas [(2.8±0.1) days vs. (3.4±0.2) days, t=0.532, P=0.030], earlier postoperative feeding [(3.6±0.2) days vs. (4.3±0.2) days, t=0.508, P=0.020], shorter drainage tube removal time [(4.2±0.4) days vs. (5.7±0.5) days, t=0.508, P=0.020] and postoperative hospital stay [(8.3±0.6) days vs. (10.7±0.3) days, t=0.502, P=0.006] as compared to the open group. (4) Great curvature of the stomach: the laparoscopy group presented less intraoperative blood loss [<100 ml ratio: 52.7% (39/74) vs. 36.5% (27/74), Z=7.681, P=0.032], earlier gastric tube removal [(2.6±0.2) days vs. (3.6±0.2) days, t=0.501, P=0.001], earlier postoperative passage of gas [(2.7±0.2) days vs. (3.4±0.2) days, t=0.501, P=0.016], earlier postoperative feeding [(3.6±0.2) days vs. (4.7±0.2) days, t=0.500, P=0.001], shorter drainage tube removal time [(4.0±0.5) days to (5.9±0.4) days, t=0.508, P=0.002] and postoperative hospital stay [(7.5±0.3) days to (9.5±0.1) days, t=0.500, P=0.001] than the open group. Subgroup analysis according to tumor size: (1) Tumor long diameter 2.0-5.0 cm: the laparoscopy group had earlier passage of gas [(2.9±0.1) days vs. (3.5±0.1) days, t=0.500, P=0.001], earlier postoperative feeding [(4.5±0.1) days vs. (5.0±0.2) days, t=0.501, P=0.013], shorter drainage tube removal time [(4.8±0.3) days vs. (6.0±0.3) days, t=0.511, P=0.008] and postoperative hospital stay [(8.1±0.4) days to (10.1±0.3) days, t=0.513, P=0.001] than the open group. (2) Tumor long diameter 5.1-10.0 cm: in the laparoscopic group, postoperative feeding time [(4.0±0.2) days vs. (4.7±0.2) days, t=0.506, P=0.015], drainage tube removal time [(4.6±0.4) days vs. (6.4±0.5)) days, t=0.501, P=0.004], postoperative hospital stay [(8.2±0.3) days vs. (10.9±0.6) days, t=0.500, P=0.001] were all shorter than those in the open group. No intraoperative and postoperative complications were observed in each group. The 5-year recurrence-free survival rates of the laparoscopy group and the open group were 95.4% and 91.6%, respectively (P=0.734), and the 5-year overall survival rates were 93.8% and 90.8% (P=0.691), respectively, and the differences were not statistically significant. Conclusions: In experienced medical centers, laparoscopic surgery for gastric GIST larger than 2 cm is safe and feasible, and can achieve comparable efficacy with open surgery. For gastric GISTs which do not locate in the greater curvature and the anterior wall of the stomach, and whose long diameter is ≤5 cm, laparoscopic surgery does not increase the risk of recurrence and metastasis, and can accelerate postoperative recovery.


Assuntos
Gastrectomia/métodos , Tumores do Estroma Gastrointestinal/cirurgia , Neoplasias Gástricas/cirurgia , Idoso , Feminino , Gastrectomia/efeitos adversos , Gastrectomia/estatística & dados numéricos , Tumores do Estroma Gastrointestinal/patologia , Humanos , Laparoscopia , Laparotomia , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Pontuação de Propensão , Estudos Retrospectivos , Neoplasias Gástricas/patologia , Resultado do Tratamento
6.
Zhonghua Wei Chang Wai Ke Za Zhi ; 23(9): 896-903, 2020 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-32927515

RESUMO

Objective: At present, the modified NIH classification commonly used in clinical practice is still insufficient for assessing the risk of postoperative recurrence in some patients with intermediate-high risk gastrointestinal stromal tumors (GIST). Through exploring risk factors for recurrence of intermediate-high risk GIST, this study establishes a predictive model for recurrence with more convenience and more precision in order to guide adjuvant therapy for intermediate-high risk GIST patients. Methods: A retrospective case-control study was carried out. Clinical and pathological data of 432 GIST patients who did not receive preoperative targeted treatment, underwent complete resection in the Union Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 2005 to June 2018, and were diagnosed as intermediate- or high-risk based on modified NIH classification by postopertive pathology, were retrospectively analyzed. Cox regression model was used to idenitify independent risk factors of recurrence, and a recurrence risk scoring model was established. The receiver operating characteristic curve (ROC curve), consistency index (C-index) and calibration curve were used to evaluate the accuracy of the scoring model in predicting the recurrence of moderate-risk and high-risk GIST patients. Results: Among 432 GIST patients, 332 were diagnosed as high-risk and 100 as moderate-risk; 237 were males and 195 females with average age of (57.4±12.4) years. Of 432 patients, 211 cases (48.8%) had fibrinogen (FIB) >3.5 g/L; 85 cases (19.7%) had platelet to lymphocyte ratio (PLR)>272.5; 122 cases (28.2%) had neutrophil to lymphocyte ratio (NLR) > 4.2; 102 cases (23.6%) had systemic inflammatory reaction index (SIRI)> 2.7; 198 cases (45.8%) had tumor long diameter >8 cm and 108 cases (25.0%) had mitotic counts > 8/50 HPF. Cox multivariable analysis showed that FIB (HR=1.789, 95% CI: 1.058-3.027, P=0.030), PLR (HR=1.862, 95% CI: 1.067-3.249, P=0.029), SIRI (HR=1.790, 95% CI: 1.039-3.084, P=0.036), tumor long diameter (HR=1.970, 95% CI: 1.105-2.925, P=0.017) and mitotic counts (HR=2.187, 95% CI:1.211-3.950, P=0.009) were independent risk factors for recurrence in patients with middle-risk and high-risk GIST. These 5 factors were included in the risk scoring model, which was given a weight score of 58 points, 62 points, 58 points, 63 points, and 78 points, respectively. Patients with a total score of ≤ 78 points were classified as moderate-risk recurrence (group I), those of 78 to 136 points as high-risk recurrence (group II) and those of >136 points as very high-risk recurrence (group III). ROC curve showed that the area under the curve (AUC) of the scoring model was 0.730 and the C-index was 0.724 (95% CI:0.687-0.787). The calibration curves and the Kaplan-Meier curves of patients in the three groups revealed that this model had a good predictive accuracy. Conclusions: For intermediate-risk and high-risk GIST patients, the preoperative FIB >3.5 g/L, PLR > 272.5 and SIRI > 2.7 are independent risk factors of recurrence after surgery. The recurrence risk scoring model established by combining tumor long diameter, mitotic counts, FIB, PLR and SIRI can effectively predict the risk of postoperative recurrence and metastasis in moderate-risk and high-risk GIST patients.


Assuntos
Fibrinogênio/análise , Neoplasias Gastrointestinais/sangue , Tumores do Estroma Gastrointestinal/sangue , Inflamação/sangue , Recidiva Local de Neoplasia/sangue , Idoso , Estudos de Casos e Controles , Terapia Combinada , Feminino , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/terapia , Humanos , Inflamação/diagnóstico , Inflamação/patologia , Inflamação/terapia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco
7.
Zhonghua Wei Chang Wai Ke Za Zhi ; 23(9): 917-921, 2020 Sep 25.
Artigo em Chinês | MEDLINE | ID: mdl-32927519

RESUMO

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumors in the gastrointestinal tract. Surgical resection is the only curative treatment, while imatinib is the first-line therapy for recurrent, metastatic, and unresectable GIST. However, more than half of GIST patients suffer from secondary resistance to imatinib within 2 years after treatment initiation. Therefore, early diagnosis, drug resistance and recurrence surveillance are critical for GIST patients. Liquid biopsy is a new method which utilizes the detection of tumor biomarkers in peripheral blood for early diagnosis and therapeutic efficacy assessment. In recent years, liquid biopsy has achieved significant research progress in several kinds of malignancy. This review aims at presenting an overview on research advance of liquid biopsy in GIST and may provide a new method for early diagnosis and therapeutic efficacy assessment of GIST.


Assuntos
Detecção Precoce de Câncer/métodos , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Biópsia Líquida , Recidiva Local de Neoplasia/diagnóstico , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Neoplasias Gastrointestinais/sangue , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/tratamento farmacológico , Tumores do Estroma Gastrointestinal/sangue , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Humanos , Mesilato de Imatinib/efeitos adversos , Mesilato de Imatinib/uso terapêutico , Recidiva Local de Neoplasia/etiologia
8.
Surgery ; 168(4): 695-700, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32713755

RESUMO

BACKGROUND: The utility of adjuvant systemic therapy in small bowel gastrointestinal stromal tumor remains unclear. METHODS: We queried the National Cancer Data Base for individuals having enterectomy to negative margins for small bowel gastrointestinal stromal tumor between 2010 and 2015. Subjects were categorized by tumor size (2.1-5 cm, 5.1-10 cm, >10 cm) and histologic grade (≤5 mitoses/50 high-power field and >5 mitoses/50 high-power field). Cox proportional hazard analysis was performed to evaluate the association between adjuvant therapy and overall survival. RESULTS: One thousand five hundred fifty-nine patients met the inclusion criteria. On univariate comparison to resection alone, adjuvant therapy was associated with improved overall survival for individuals with high-grade tumors of intermediate and large size (85% vs 48%, P = .010; 75% vs 47%, P = .003) but not for those with high-grade tumors of small size or low-grade tumors of any size. On multivariable analysis adjusted for age, comorbid disease state, and tumor size, adjuvant therapy was independently associated with reduced risk of mortality for high-grade (hazard ratio 0.37, 95% confidence interval: 0.21-0.64) but not low-grade tumors. CONCLUSION: Adjuvant therapy after R0 resection for small bowel gastrointestinal stromal tumor should be administered after careful consideration of the size and grade of a patient's tumor.


Assuntos
Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/cirurgia , Mesilato de Imatinib/uso terapêutico , Neoplasias Intestinais/tratamento farmacológico , Neoplasias Intestinais/cirurgia , Intestino Delgado/cirurgia , Idoso , Quimioterapia Adjuvante , Feminino , Tumores do Estroma Gastrointestinal/patologia , Humanos , Neoplasias Intestinais/patologia , Intestino Delgado/patologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Modelos de Riscos Proporcionais , Estudos Retrospectivos
9.
Lancet Oncol ; 21(7): 935-946, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32615108

RESUMO

BACKGROUND: Targeting of KIT and PDGFRA with imatinib revolutionised treatment in gastrointestinal stromal tumour; however, PDGFRA Asp842Val (D842V)-mutated gastrointestinal stromal tumour is highly resistant to tyrosine kinase inhibitors. We aimed to assess the safety, tolerability, and antitumour activity of avapritinib, a novel KIT and PDGFRA inhibitor that potently inhibits PDGFRA D842V, in patients with advanced gastrointestinal stromal tumours, including patients with KIT and PDGFRA D842V-mutant gastrointestinal stromal tumours (NAVIGATOR). METHODS: NAVIGATOR is a two-part, open-label, dose-escalation and dose-expansion, phase 1 study done at 17 sites across nine countries (Belgium, France, Germany, Poland, Netherlands, South Korea, Spain, the UK, and the USA). Patients aged 18 years or older, with an Eastern Cooperative Oncology Group performance status of 2 or less, and with adequate end-organ function were eligible to participate. The dose-escalation part of the study included patients with unresectable gastrointestinal stromal tumours. The dose-expansion part of the study included patients with an unresectable PDGFRA D842V-mutant gastrointestinal stromal tumour regardless of previous therapy or gastrointestinal stromal tumour with other mutations that either progressed on imatinib and one or more tyrosine kinase inhibitor, or only received imatinib previously. On the basis of enrolment trends, ongoing review of study data, and evolving knowledge regarding the gastrointestinal stromal tumour treatment paradigm, it was decided by the sponsor's medical director together with the investigators that patients with PDGFRA D842V mutations would be analysed separately; the results from this group of patients is reported in this Article. Oral avapritinib was administered once daily in the dose-escalation part (starting dose of 30 mg, with increasing dose levels once daily in continuous 28-day cycles until the maximum tolerated dose or recommended phase 2 dose was determined; in the dose-expansion part, the starting dose was the maximum tolerated dose from the dose-escalation part). Primary endpoints were maximum tolerated dose, recommended phase 2 dose, and safety in the dose-escalation part, and overall response and safety in the dose-expansion part. Safety was assessed in all patients from the dose-escalation part and all patients with PDGFRA D842V-mutant gastrointestinal stromal tumour in the dose-expansion part, and activity was assessed in all patients with PDGFRA D842V-mutant gastrointestinal stromal tumour who received avapritinib and who had at least one target lesion and at least one post-baseline disease assessment by central radiology. This study is registered with ClinicalTrials.gov, NCT02508532. FINDINGS: Between Oct 26, 2015, and Nov 16, 2018 (data cutoff), 46 patients were enrolled in the dose-escalation part, including 20 patients with a PDGFRA D842V-mutant gastrointestinal stromal tumour, and 36 patients with a PDGFRA D842V-mutant gastrointestinal stromal tumour were enrolled in the dose-expansion part. At data cutoff (Nov 16, 2018), 38 (46%) of 82 patients in the safety population (median follow-up of 19·1 months [IQR 9·2-25·5]) and 37 (66%) of the 56 patients in the PDGFRA D842V population (median follow-up of 15·9 months [IQR 9·2-24·9]) remained on treatment. The maximum tolerated dose was 400 mg, and the recommended phase 2 dose was 300 mg. In the safety population (patients with PDGFRA D842V-mutant gastrointestinal stromal tumour from the dose-escalation and dose-expansion parts, all doses), treatment-related grade 3-4 events occurred in 47 (57%) of 82 patients, the most common being anaemia (14 [17%]); there were no treatment-related deaths. In the PDGFRA D842V-mutant population, 49 (88%; 95% CI 76-95) of 56 patients had an overall response, with five (9%) complete responses and 44 (79%) partial responses. No dose-limiting toxicities were observed at doses of 30-400 mg per day. At 600 mg, two patients had dose-limiting toxicities (grade 2 hypertension, dermatitis acneiform, and memory impairment in patient 1, and grade 2 hyperbilirubinaemia in patient 2). INTERPRETATION: Avapritinib has a manageable safety profile and has preliminary antitumour activity in patients with advanced PDGFRA D842V-mutant gastrointestinal stromal tumours. FUNDING: Blueprint Medicines.


Assuntos
Neoplasias Gastrointestinais/tratamento farmacológico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Mutação , Pirazóis/uso terapêutico , Pirróis/uso terapêutico , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética , Triazinas/uso terapêutico , Idoso , Feminino , Seguimentos , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico
11.
Lancet Oncol ; 21(7): 923-934, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32511981

RESUMO

BACKGROUND: Resistance to approved inhibitors of KIT proto-oncogene, receptor tyrosine kinase (KIT), and platelet-derived growth factor receptor α (PDGFRA) is a clinical challenge for patients with advanced gastrointestinal stromal tumours. We compared the efficacy and safety of ripretinib, a switch-control tyrosine kinase inhibitor active against a broad spectrum of KIT and PDGFRA mutations, with placebo in patients with previously treated, advanced gastrointestinal stromal tumours. METHODS: In this double-blind, randomised, placebo-controlled, phase 3 study, we enrolled adult patients in 29 specialised hospitals in 12 countries. We included patients aged 18 years or older who had advanced gastrointestinal stromal tumours with progression on at least imatinib, sunitinib, and regorafenib or documented intolerance to any of these treatments despite dose modifications, and who had an Eastern Cooperative Oncology Group (ECOG) performance status of 0-2. Eligible patients were randomly assigned (2:1) to receive either oral ripretinib 150 mg once daily (ripretenib group) or placebo once daily (placebo group). Randomisation was done via an interactive response system using randomly permuted block sizes of six and stratified according to number of previous therapies and ECOG performance status. Patients, investigators, research staff, and the sponsor study team were masked to a patient's treatment allocation until the blinded independent central review (BICR) showed progressive disease for the patient. The primary endpoint was progression-free survival, assessed by BICR. The primary analysis was done in the intention-to-treat population and safety was assessed in patients who received at least one dose of study drug. Patients randomly assigned to placebo were permitted to cross over to ripretinib 150 mg at the time of disease progression. The INVICTUS study is registered with ClinicalTrials.gov, number NCT03353753, and with WHO International Clinical Trials Registry Platform, number EUCTR2017-002446-76-ES; follow-up is ongoing. FINDINGS: Between Feb 27, 2018, and Nov 16, 2018, 129 of 154 assessed patients were randomly assigned to receive either ripretinib (n=85) or placebo (n=44). At data cutoff (May 31, 2019), at a median follow-up of 6·3 months (IQR 3·2-8·2) in the ripretinib group and 1·6 months (1·1-2·7) in the placebo group, 51 patients in the ripretinib group and 37 in the placebo group had had progression-free survival events. In the double-blind period, median progression-free survival was 6·3 months (95% CI 4·6-6·9) with ripretinib compared with 1·0 months (0·9-1·7) with placebo (hazard ratio 0·15, 95% CI 0·09-0·25; p<0·0001). The most common (>2%) grade 3 or 4 treatment-related treatment-emergent adverse events in the ripretinib group (n=85) included lipase increase (four [5%]), hypertension (three [4%]), fatigue (two [2%]), and hypophosphataemia (two (2%]); in the placebo group (n=43), the most common (>2%) grade 3 or 4 treatment-related treatment-emergent adverse events were anaemia (three [7%]), fatigue (one [2%]), diarrhoea (one [2%]), decreased appetite (one [2%]), dehydration (one [2%]), hyperkalaemia (one [2%]), acute kidney injury (one [2%]), and pulmonary oedema (one [2%]). Treatment-related serious adverse events were reported in eight (9%) of 85 patients who received ripretinib and three (7%) of 43 patients who received placebo. Treatment-related deaths occurred in one patient in the placebo group (septic shock and pulmonary oedema) and one patient in the ripretinib group (cause of death unknown; the patient died during sleep). INTERPRETATION: Ripretinib significantly improved median progression-free survival compared with placebo and had an acceptable safety profile in patients with advanced gastrointestinal stromal tumours who were resistant to approved treatments. FUNDING: Deciphera Pharmaceuticals.


Assuntos
Neoplasias Gastrointestinais/tratamento farmacológico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Compostos de Fenilureia/uso terapêutico , Piridinas/uso terapêutico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Método Duplo-Cego , Feminino , Seguimentos , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Taxa de Sobrevida , Adulto Jovem
12.
Int J Clin Oncol ; 25(8): 1506-1514, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32577952

RESUMO

BACKGROUND: Improved prediction of prognosis for gastrointestinal stromal tumours (GISTs) has become increasingly important since the introduction of targeted therapy. Here, we aimed to evaluate the prognostic significance of preoperative plasma fibrinogen (Fib) levels in patients with primary GISTs and to analyse their correlations with clinicopathological characteristics. METHODS: A total of 201 previously untreated patients with primary GISTs who had undergone radical surgery at our institution between October 2004 and July 2018 were enrolled. The optimal cut-off value for Fib levels was calculated using time-dependent receiver-operating characteristic curve analysis. RFS, the primary endpoint, was calculated by the Kaplan-Meier method and compared by the log-rank test. Univariate and multivariate Cox regression models were calculated. RESULTS: High preoperative plasma Fib levels were detected as an independent adverse prognostic factor (p = 0.008, hazard ratio 3.136, 95% CI 1.356‒7.256). Furthermore, high preoperative plasma Fib levels also indicated a poor prognosis within the modified National Institutes of Health (mNIH) high-risk subgroup (p = 0.041). In addition, preoperative plasma Fib levels showed a positive correlation with several prognostic factors and even a linear relationship with tumour size (Spearman correlation coefficient [r] = 0.411, p < 0.001). CONCLUSIONS: Our results suggest that high preoperative plasma Fib levels may indicate a poor prognosis in patients with primary GISTs. As a cost-effective biomarker, preoperative assessment of plasma Fib levels may help to further risk stratify patients with mNIH high-risk GISTs and instruct the application of targeted therapy.


Assuntos
Fibrinogênio/análise , Tumores do Estroma Gastrointestinal/sangue , Tumores do Estroma Gastrointestinal/mortalidade , Tumores do Estroma Gastrointestinal/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Feminino , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Período Pré-Operatório , Prognóstico , Modelos de Riscos Proporcionais , Curva ROC , Estudos Retrospectivos
13.
Mol Carcinog ; 59(6): 661-669, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32339330

RESUMO

Gastrointestinal stromal tumor (GIST) is a common mesenchymal tumor in the gastrointestinal tract. Prostate cancer associated transcript 6 (PCAT6) is a long noncoding RNA (lncRNA) and plays a pivotal role in tumor formation. Present study was designed to explore the function of PCAT6 in GIST. Ki67 staining, colony formation and trypan blue staining assays revealed that PCAT6 boosted GIST cell proliferation but inhibited cell apoptosis. Also, sphere formation assay and Western blot uncovered the promoting role of PCAT6 in GIST stemness. Then, we identified that PCAT6 could activate Wnt/ß-catenin pathway. And the tumor facilitator role of Wnt/ß-catenin pathway was validated in the rescue assays. Next, miR-143-3p was identified as the downstream microRNA of PCAT6. Moreover, miR-143-3p itself served as a tumor suppressor in GIST. Subsequently, peroxiredoxin 5 (PRDX5) was verified as the target of miR-143-3p. PCAT6 promoted GIST cell proliferation and stemness via sponging miR-143-3p to upregulate PRDX5. In a word, PCAT6 promoted GIST cell proliferation and stemness but inhibited cell apoptosis via competing endogenous RNA pattern and activation of Wnt pathway, which might contribute to GIST treatment.


Assuntos
Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/patologia , Regulação Neoplásica da Expressão Gênica , MicroRNAs/genética , Peroxirredoxinas/metabolismo , RNA Longo não Codificante/genética , Via de Sinalização Wnt , Apoptose , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Proliferação de Células , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/metabolismo , Tumores do Estroma Gastrointestinal/genética , Tumores do Estroma Gastrointestinal/metabolismo , Humanos , Células-Tronco Neoplásicas/metabolismo , Células-Tronco Neoplásicas/patologia , Peroxirredoxinas/genética , Prognóstico , Ativação Transcricional , Células Tumorais Cultivadas , beta Catenina/genética , beta Catenina/metabolismo
14.
Medicine (Baltimore) ; 99(17): e18799, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32332594

RESUMO

RATIONALE: Fibromatoses or desmoid tumors are relatively rare tumors derived from the musculoaponeurotic system. This tumor has no specific clinical symptoms and it is sometimes misdiagnosed as other diseases such as gastrointestinal stromal tumors (GISTs). PATIENT CONCERNS: A 28-year-old man visited Peking Union Medical College for a tangible abdominal mass without abdominal pain or distention. DIAGNOSES: Considering the imaging characteristics and clinical manifestation, this mass was primarily diagnosed as GIST before surgery. During the surgery, the occupancy was found under the ileocecal mesentery, with grayish white appearance, tough texture, and poor mobility, which was not consistent with the character of the GIST. After the surgery, pathological examination and individual immunohistochemistry results demonstrated that the lesion was compatible with the diagnosis of retroperitoneal fibromatosis with purulent inflammation of chronic lymphadenitis. INTERVENTIONS: Therefore, we decided to perform tumor mass resection, right colon resection, partial duodenum resection, and intestinal anastomosis on laparotomy, but the right ureter was retained. After excision of the tumor, the ends of the intestine segment were continuously sutured. OUTCOMES: The patient experienced no intraoperative or postoperative complications, and was discharged 3 days after surgery. Periodic follow-up physical examinations such as the abdominal ultrasound and computed tomography were performed each 3 months, and no evidence of recurrence was observed during the whole 12 months. LESSONS: To sum up, intra-abdominal fibromatosis is an extremely rare tumor that must be differentiated from other tumors of the digestive tract, and pathological and immunohistochemical examination is a critical part of the diagnosis. Early diagnosis of fibromatosis is essential for the outcome. Extensive resection of the mass minimizes the risk of relapse.


Assuntos
Fibromatose Abdominal/diagnóstico , Fibromatose Abdominal/patologia , Espaço Retroperitoneal/patologia , Adulto , China , Diagnóstico Diferencial , Fibromatose Abdominal/diagnóstico por imagem , Fibromatose Abdominal/cirurgia , Tumores do Estroma Gastrointestinal/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Tomografia Computadorizada por Raios X
15.
Medicine (Baltimore) ; 99(16): e19885, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32312014

RESUMO

RATIONALE: Gastrointestinal stromal tumor (GIST) is one of the most common malignant mesenchymal tumors of the gastrointestinal tract. They generally arise from the fourth layer (muscularis propria) and rarely from the second or third layer. Although the manifestations of gastric stromal tumors are diverse, to our knowledge, there are only several cases of an extra-gastric stromal tumor in the literature appearing with a pedunculation. Pedunculated large GISTs are not frequent and compress the neighboring organs. When they were huge, it is difficult to differentiate the origin of the masses. Thus, in the clinical setting, physicians should pay more attention to the pattern of manifestation of the gastric stromal tumor. PATIENT CONCERNS: A 62-year-old man had no gastrointestinal symptoms or significant medical and family histories. During the health examination with US, a cystic-solid tumor was found below liver. The results of the physical examination were unremarkable, and routine laboratory data on admission did not show any abnormal findings. DIAGNOSIS: Computed tomography of the abdomen showed a mixed echoic mass measuring 10 × 8 × 8 cm and located below the liver, adjacent to the gastric antrum. After endoscopic ultrasound-guided fine-needle aspiration, cytopathology showed that the specimen was filled with red blood cells, and it had no malignant cells. Histopathology revealed that the mass was a GIST, and immunohistochemical analysis showed the following: CD117(+), CD34(+), desmin(-), Dog-1(+), Ki-67% <1%, and smooth muscle actin(-). INTERVENTIONS: Surgical resection was performed on the patient. OUTCOMES: The lesion was diagnosed as a gastric stromal tumor with a pedicle and an old hemorrhage. The patient's recovery was uneventful. After surgery, computed tomography at the 6-month and 1-year postoperative follow-up visits did not reveal relapse or any metastasis. LESSONS: In the clinical setting, physicians should pay more attention to the pattern of manifestation of the extra-gastric stromal tumor in patients with a pedicle or hemorrhage. Additionally, endoscopic ultrasound-guided fine-needle aspiration can be used to make an accurate preoperative diagnosis of such diseases, and its findings can serve as an important basis for surgical excision of the lesions.


Assuntos
Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Neoplasias Gástricas/patologia , Estômago/patologia , Assistência ao Convalescente , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Proteínas Proto-Oncogênicas c-kit/metabolismo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
16.
BMC Surg ; 20(1): 42, 2020 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-32122361

RESUMO

BACKGROUND: We herein report a rare case of an ectopic bronchogenic cyst of the gastric cardia. The initial diagnosis was a gastrointestinal stromal tumor (GIST); however, postoperative pathologic examination confirmed that it was a bronchogenic cyst. CASE PRESENTATION: A 62-year-old woman visited our hospital for abdominal pain. The diagnosis prior to surgery was a GIST. Computed tomography imaging showed that the mass was located in the gastric cardia on the side of the lesser curvature. During the surgical exploration, it was noted that the tactility of the mass was not consistent with a GIST. Thus, we decided to perform local resection of the mass and part of the gastric wall without wedge resection. The pathological examination revealed a bronchogenic cyst. CONCLUSIONS: This case suggests that a bronchogenic cyst should be considered as a differential diagnosis of a GIST. It is also a unusual but necessary situation should be considered when explaining the etiology of a bronchogenic cyst.


Assuntos
Cisto Broncogênico/diagnóstico , Tumores do Estroma Gastrointestinal/diagnóstico , Neoplasias Gástricas/diagnóstico , Dor Abdominal/etiologia , Cárdia/patologia , Diagnóstico Diferencial , Feminino , Tumores do Estroma Gastrointestinal/patologia , Humanos , Pessoa de Meia-Idade , Neoplasias Gástricas/cirurgia , Tomografia Computadorizada por Raios X
17.
Medicine (Baltimore) ; 99(9): e19275, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32118738

RESUMO

The advent of imatinib mesylate (IM) has dramatically revolutionized the prognosis of advanced and metastatic/recurrent gastrointestinal stromal tumors (GISTs). The objective of this retrospective study is to investigate the safety and efficacy of combination of surgery following IM treatment in the management of advanced and metastatic/recurrent GISTs. We further explore the long-term clinical outcomes in these who underwent therapy of preoperative IM.Eligible patients with GISTs before the onset of the IM therapy and were periodically followed up in the outpatient clinic were included in this study. Detailed clinical and pathologic characteristics were obtained from the medical records of our institution. Univariate and multivariate regression analyses were performed to use for the evaluation of potential prognostic factors.A total of 51 patients were included in the study, of these patients, 36 patients underwent surgery and median duration of preoperative IM is 8.2months (range 3.5-85 months). Significant median tumor shrinkage rate was 29.27% (95% confidence interval 21.00%-34.00%) observed in these patients who responded to IM, and partial response and stable disease were achieved in 24 patients (47.06%) and 23 patients (45.10%), respectively, in light of the RECIST guideline (version 1.1). After the median follow-up of 43.70 months (range 14.2-131.1 months), 1- and 3-year overall survival (OS) were estimated to be 96.1% and 94.0%, respectively, and there was a significant improvement in OS for patients who received surgical intervention versus those who did not.Our study consolidates that patients were received preoperative IM therapy could shrink the size of tumors and facilitate organ-function preservation. The long-term analysis on this study supports that surgical intervention following IM therapy benefits for patients with primary advanced and recurrent or metastatic GISTs on long-term prognosis.


Assuntos
Antineoplásicos/uso terapêutico , Neoplasias Gastrointestinais/terapia , Tumores do Estroma Gastrointestinal/terapia , Mesilato de Imatinib/uso terapêutico , Recidiva Local de Neoplasia/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/administração & dosagem , China , Feminino , Neoplasias Gastrointestinais/diagnóstico por imagem , Neoplasias Gastrointestinais/mortalidade , Neoplasias Gastrointestinais/patologia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/mortalidade , Tumores do Estroma Gastrointestinal/patologia , Humanos , Mesilato de Imatinib/administração & dosagem , Masculino , Registros Médicos , Pessoa de Meia-Idade , Terapia Neoadjuvante , Metástase Neoplásica , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
18.
Medicine (Baltimore) ; 99(9): e19346, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32118771

RESUMO

RATIONALE: Available literature states that the histological subtype of the gastrointestinal stromal tumor (GIST) with pulmonary metastasis is often spindle cell type. To our knowledge, this is the first report of the GIST with pulmonary metastasis of very uncommon epithelioid subtype. PATIENT CONCERNS: We report a 63-year-old male presenting with the symptom of bloodstained sputum without obvious inducement. The patient had no chest pain, low back pain, fatigue, fever or night sweats symptoms. DIAGNOSES: Combined chest digital radiography and the history of the patient who presented with the colon GIST of the epithelioid subtype two years ago that the mass may be a metastasis tumor. Combined with morphological and immunohistochemical staining results, a pathological diagnosis of the GIST with pulmonary metastasis was considered. INTERVENTIONS: Right lobectomy and partial upper lobectomy were performed. OUTCOMES: The patient had not experienced any noticeable symptom and recurrent tumors at 6 months follow-up. LESSONS: We report a rare case of the GIST with pulmonary metastasis of epithelioid subtype. This case is of great significance to the pathologist's clinical work. For pathologists, if an epithelioid tumor in the lung is found, it is necessary to check whether the gastrointestinal tract also has the tumor, which may be an epithelioid GIST with pulmonary metastasis.


Assuntos
Tumores do Estroma Gastrointestinal/complicações , Neoplasias Pulmonares/etiologia , Biomarcadores Tumorais/análise , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/fisiopatologia , Humanos , Pulmão/anormalidades , Pulmão/patologia , Neoplasias Pulmonares/fisiopatologia , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica/patologia , Metástase Neoplásica/fisiopatologia
19.
Rev. esp. patol ; 53(1): 37-41, ene.-mar. 2020. ilus
Artigo em Espanhol | IBECS | ID: ibc-195574

RESUMO

Actualmente, la mayoría de las neoplasias presentes en el estómago son de tipo epitelial, constituyendo el subtipo más frecuente. El siguiente subtipo de tumor más común es el estromal, siendo el tumor del estroma gastrointestinal el más frecuente, seguido por el leiomioma y el schwannoma. En el presente artículo mostramos el caso excepcional de un paciente con un tumor del estroma gastrointestinal y sospecha de enfermedad residual gástrica, la cual fue diagnosticada posteriormente como schwannoma tras su exéresis


The majority of gastric neoplasms are of epithelial type. Stromal tumours are the next most frequent and are most commonly gastrointestinal stromal tumours, followed by leiomyoma and schwannoma. We present an exceptional case of a patient with a gastrointestinal stromal tumour with suspicion of residual gastric disease, which was diagnosed post-operatively as a schwannoma


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Tumores do Estroma Gastrointestinal/diagnóstico , Neurilemoma/diagnóstico , Neurofibromatoses/diagnóstico , Tumores do Estroma Gastrointestinal/patologia , Neurilemoma/patologia , Neurofibromatoses/patologia
20.
J. coloproctol. (Rio J., Impr.) ; 40(1): 12-19, Jan.-Mar. 2020. tab, graf, ilus
Artigo em Inglês | LILACS | ID: biblio-1090846

RESUMO

Abstract Background This study defines the disease profile in south Indian population and determine the clinic-pathological aspects of Gastro-Intestinal Stromal Tumors. Method In this prospective study patients diagnosed of gastrointestinal stromal tumors were taken thorough clinical examination and a database of Anthropometric details and clinical details were analyzed. Pathological data included tumor size, presence or absence necrosis, mitotic counts, immunohistochemistry for CD-117, CD-34. Results There were 44 patients with confirmed diagnosis of gastro-intestinal stromal tumor. The highest incidence was found in the 6th decade. The most common symptoms were abdominal pain and gastrointestinal bleed. Stomach was most frequent site for gastro-intestinal stromal tumors. Immunochemistry for CD-117 was positive in 93.18% cases. Majority of tumors (79.5%) had pure spindle cell morphology and mitotic activity showed that 34% of the GISTs were of the high risk group. Forty two patients were suggestive of surgery as the primary treatment after presentation. Conclusion Abdominal pain was the most common presenting complaint. Majority of the tumors aroused from the stomach. The majority of the tumors had pure spindle cell morphology and 93% of the tumors were CD-117 positive. A significant relationship between tumor size, tumor necrosis and mitotic activity with large tumors having necrosis and high mitotic rate having high risk of malignancy, was observed. Surgical resection is considered mainstay of treatment of gastro-intestinal stromal tumor. Imatinib therapy should be given to patients in moderate to severe risk categories.


Resumo Justificativa Este estudo define o perfil da doença na população do sul da Índia e determina os aspectos clínicos e patológicos dos tumores estromais gastrointestinais. Método Neste estudo prospectivo, os pacientes diagnosticados com tumor estromal gastrointestinl foram submetidos a um exame clínico completo, e uma série de dados dos pacientes, incluindo detalhes antropométricos e clínicos, foram analisados. Os dados patológicos incluíram tamanho do tumor, presença ou ausência de necrose, contagem mitótica e imuno-histoquímica para CD-117, CD-34. Resultados Havia 44 pacientes com diagnóstico confirmado de tumor estromal gastrointestinal. A maior incidência foi encontrada na 6ª década de vida. Os sintomas mais comuns foram dor abdominal e sangramento gastrointestinal. O estômago foi o local mais frequente para tumores estromais gastrointestinais. A imuno-histoquímica para CD-117 foi positiva em 93,18% dos casos. A maioria dos tumores (79,5%) apresentava morfologia pura de células fusiformes e a atividade mitótica mostrou que 34% dos GISTs pertenciam ao grupo de alto risco. Quarenta e dois pacientes receberam indicação para cirurgia como tratamento primário após a apresentação. Conclusão A dor abdominal foi a queixa mais comum. A maioria dos tumores afetava o estômago, apresentava morfologia pura de células fusiformes e 93% eram CD-117 positivos. Foi observada uma relação significativa entre o tamanho do tumor, a necrose tumoral e a atividade mitótica, com os tumores grandes apresentando necrose e alta taxa mitótica com alto risco de malignidade. A ressecção cirúrgica é considerada o principal tratamento do tumor estromal gastrointestinal. A terapia com imatinibe deve ser administrada a pacientes em categoria de risco de moderadas a grave.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Tumores do Estroma Gastrointestinal/patologia , Neoplasias Gastrointestinais , Proteínas Proto-Oncogênicas c-kit/imunologia , Antígenos CD34/imunologia , Mesilato de Imatinib/uso terapêutico , Índia , Antineoplásicos/uso terapêutico
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